USMLE Heme Onc Flashcards
1
Q
Acute leukemia
A
Thrombocytopenia
2
Q
AL amyloidosis dx
A
In patients with suspected AL amyloidosis, the combination of bone marrow biopsy and abdominal fat pad aspiration has a sensitivity of approximately 90%.
classic features of AL amyloidosis, including macroglossia, hepatomegaly, nephrotic syndrome, peripheral neuropathy, and the presence of an IgG lambda M-protein
3
Q
Acute Lymphocytic Leukemia
Prognostic factors:
A
- Age — the young fare better (< 30)
- Blast count > 30,000 do poorly
- Cytogenetics — hyperdiploidy is a good finding, whereas a Philadelphia chromosome t(9,22) is a poor prognostic finding
4
Q
Acute Lymphocytic Leukemia
Tx
A
- Supportive care with transfusions
- Leukophoresis is not usually necessary since the blasts are smaller and less likely to cause sludging
- Combination chemotherapy
- Induction chemotherapy using multi-drug regimens
- Consolidation chemotherapy with multi-drug regimens for multiple cycles
- Maintenance (different from Myeloid)
- CNS prophylasxis intrathecal / radiation
5
Q
Alpha Thalassemia
A
- aaa- -a-a- -a— —
- (Hgb H) (Hgb Barts)
- Should have a low MCV
- Should have an elevated reticulocyte count if anemic
- Normal electrophoresis
- Diagnosis can be made by a chain gene analysis
6
Q
AML M3
- Cytogenetics = t(15,17)
- Tx:
A
**_AML /// M3 /// DIC _ **
- Translocation involving the retinoic acid receptor gene
- Good prognosis category
- Prominent Auer rods
- Commonly associated with DIC
- Tx: All Trans Retinoic Acid (ATRA) + chemotherapy with at least an anthracycline
7
Q
AML Treatment
- Induction
- Consolidation Chemo
A
- Supportive care with transfusions
- Leukophoresis if blast count > 100,000 or for symptoms of hyperviscosity
- Combination chemotherapy
- Bone marrow transplant
- Induction chemotherapy — designed to take a patient to aplasia with recovery of “normal” hematopoiesis and a remission state
- Consolidation chemotherapy — designed to reinforce the remission obtained; usually multiple cycles given
8
Q
Anal cancer treatment?
A
- Anal cancer is treated initially with combined radiation therapy and chemotherapy.
- Mitomycin plus 5-fluorouracil is the standard chemotherapy regimen used in conjunction with radiation therapy in the treatment of anal cancer.
9
Q
Anemia Of Inflamation
A
- Iron Low
- TIBC Low
- % sat Low/Normal
- Ferritin High ( > 100)
Usually normochromic normocytic RBCs - Decreased reticulocyte count
Gold standard - Bone Bx
10
Q
Anemia Of Inflamation Tx
A
Exogenous erythropoietin • Transfusion support • Optimize associated medical illness
11
Q
Antiphospholipid antibody
PT / PTT effect
A
PTT prolonged
12
Q
Antiphospholipid Antibody Syndrome
A
• Prolonged PTT (kinda odd eventhough its a hypercoagulable dz)
- Treatment options
- Anticoagulation
- Warfarin
- Heparin/LMWH
- ASA
- Anticoagulation
13
Q
Antithrombin III Deficiency
Tx:
A
- It Inactivates activated IX, X, XI, XII
- Should always measure p_rior to the institution of heparin therapy but may be decreased secondary to thrombosis_
- Tx: Heparin (resistant) / Warfarin long term / AT III recombinant for surgery
14
Q
(activated Protein C)
APC Resistance
Tx:
A
- Mutation in Factor V, resulting in resistance to activated protein C
- Most common inherited hypercoagulable defect
- Found in up to 25% of patients with recurrent thrombosis
- Additive to other risk factors (OCRs, pregnancy,other defects)
- Tx: Warfarin / hparin / LMWH
15
Q
Aplastic Anemia / causes
A
- Drugs
- Sulfa drugs
- Chloramphenicol
- Radiation
- Benzenes
- Gold
- PNH
- Viral infection
DX with bone marrow
16
Q
Aplastic Anemia Tx:
A
Immunosuppression with ATG [Anti-thymocyte globulin] /cyclosporine
- Older patients
- Younger patients with no marrow donor
Bone marrow transplant
- Younger patients with a donor
17
Q
Treat a postmenopausal patient with newly diagnosed breast cancer with well-differentiated, estrogen receptor-positive/progesterone receptor-positive, HER2-negative breast cancer.
A
Aromatase inhibitors are the standard of care for postmenopausal women with these tumors.
Postmenopausal women with hormone receptor–positive breast cancer should take a 5-year course of an aromatase inhibitor as primary treatment or for an additional** 5 years after completing a **5-year course of tamoxifen therapy. In women who are initially treated with tamoxifen, an aromatase inhibitor may be started following 2 to 3 years of tamoxifen therapy to complete a total of 5 years of hormonal therapy.
Trastuzumab is indicated for tumors that overexpress HER2
18
Q
Asymptomatic follicular lymphoma
A
Regardless of the stage at presentation, patients with asymptomatic follicular lymphoma can be followed without therapeutic intervention until they experience symptoms.
19
Q
A
Auer Cells / AML
20
Q
Autoimmune Hemolytic Anemia
A
- Macrocytic due to hight retic count / higher LDH / Higher Bilirubin indirect
- Direct Coombs Test
- Demonstrates antibody on the RBC surface / Mix patient’s blood with anti-IgG or C3 antibodies
- Indirect Coombs
- Test Demonstrates antibody in the serum
21
Q
- Autoimmune hemolytic anemia
- Glucose 6 phosphate dehydrogenase def
- paroxysmal nocturnal hemoglobinuria
- Pyruvate kinase def
- Microagiopathic hemolytic anemia
A
AIHA - warm antibody / spherocytes and direct coombs test
PNH: Associated with pancytopenia, hemolytic anemia and thrombosis
PK def: differs from G6PD def in that erythrocytes from PK patients are not subject to hemolysis following oxidative stress and no characteristic morphologic red cell abnormalities on peripheral smear.
Microangiopathic HA have schistocytes on peripheral blood smear and low plt
G6PD def:
22
Q
B12/Folate Deficiencies
A
- Macrocytic
- Elevated RDW
- Decreased WBC and platelet counts
- Hypersegmentation
- Decreased reticulocyte count
- *MMA** level is high in B12 Def
- *Homocysteine** is High in both
23
Q
Best strategy to prevent further CVS complications in SCD
A
Chronic simple transfusion therapy has been shown to be more effective then hydroxyurea
24
Q
Beta Thalassemia
A
- Decreased production of the 3 globin chain
- Should have a low MCV
- Should have an elevated reticulocyte count
- Should see elevated levels of Hgb A2 on hemoglobin electrophoresis
25
Bite Cells
Shistocytes (helmet cells)
Spherocytes
Target cells
Tear drop cells
Basophillic stippling
Howe Jolly
Due to phagocytes / thalassemia **/ _G6PD def_**
RBC fragemnt / **_DIC, TTP or HUS Tramatic hemolysis_**
Shperoidal RBC no central palar / Autoimmune / **_hereditary Spherocytosis_**
Target cells bulls eye / **_thalassemia_**, RbC dz, postsplenectomy or liver dz
Tear drop / **_Meylofibrosis_**, major thalasemia
Basophillic stippling / blue grannules / **_thalassemia and iron def_**, ETOH abuse , lead
Howel Jolly bodies / rbc nuclear remnant / **_asplenia**_ or _**splenic hypofunctions_**
26
Acute Lymphocytic Leukemia
* Primarily occurs in children
* Lymphadenopathy and splenomegaly occur in 50%
* An anterior mediastinal mass is common with the T-cell subtypes\*\*(young male with mediastinal mass)
* CNS disease is common\*\*
* Classification made using stains, flow cytometry, and cytogenetics
27
AE AE AE AE /// Toxicity
1. Bleomycin
2. Anthracyclines /Trastuzumab
3. Methotrexate
4. Cisplatin
5. Cyclophosphamide
6. 5 FU
1. Pulmonary fibrosis
2. Cardiomyopathy
3. Liver toxicity /pneumonitis
4. nephrotociity / ototoxicity
5. hemorrhagic cystitis / bladd canc
6. Sever diarrhea
28
Bleeding treatments in renal failure (uremic)
DDAVP
* Conjugated estrogen (second line)
* Cryoprecipitate
1. Increase release of Factor VIII/VW factor polymers from endothelial storage site
2. Possible increase platelet reactivity
3. Enhance platelet aggregation
29
Bone metastases
**_Most common would include:_**
* Prostate
* Breast
* Lung
* Renal cell
* _Myeloma (**Lytic always**)_
Diagnosis should be made with:
* Bone scan (**_shows blastic only_**)
* Metastatic bone survey
* **needed to see the lytic lesions**
Treatment should include:
* **Radiation therapy for pain control**
* Evaluation for need for prophylactic rod placement
* Consideration of bisphosphonate therapy for breast cancer and myeloma
30
Brain Metastases
Most common tumors associated are:
* Lung
* Breast
* Melanoma
* Renal cell carcinoma
Treatment includes:
* Immediate **_large doses of steroids_** usually IV dexame thasone
* If the lesion is single, resection or stereotactic treatment may be tried
* For **_multiple lesions, and after any resection_**, whole-brain radiation therapy should be given
31
Breast Cancer Risk
• BRCA1
- **_Chromosome 17: AD_**
- Lifetime risk of developing **breast cancer is 50% to 85%**
- Also increases the lifetime risk of developing **ovarian cancer to 33%**
- Associated with an increase in the risk of breast and prostate cancer in men
32
Breast Cancer
• Prevention
* The use of tamoxifen reduces the risk of developing breast cancer in patients with increased risk
* In **_High risk patients_**
* Previous cancer
* Strong family history
* BRCA1-or BRCA2-positive
33
Breast Cancer
BRCA2
- **_Chromosome 13: AD_**
- Similar risk for breast cancer 50-85%
- Somewhat smaller risk for **_ovarian cancer (10-20%)_**
- Associated with increased incidence of **breast cancer** in men
34
Breast Mass:
Solid vs. cystic??
What is next?
• Solid vs. cystic??
* Can determine using **_ultrasound_**
* **_If cystic_**, can _aspirate_; _**if bloody** fluid or recurs_, then should **_biopsy_**
* **_If solid**_, should do _**FNA or core biopsy_**
• _Palpable mas_s without abnormality on mammogram
* *_Should biopsy_*
35
Breast Cancer Treatment
* **\< 1-cm tumor**, node negative — NO ADDITIONAL TREATMENT
* **\>1 cm tumor** or (anysize tumore +node positive) —ADJUVANT TREATMENT
* **_Premenopausal_**
* chemo therapy
* +/- hormones (If ER/PR +ve then 5 yrs hormone/ **_Tamoxifen_** therapy)
* **_Postmenopausal_**
* hormones (**_aromatase inhibitors_**) +/- chemo
* chemo +/- hormones
* **_TRASTUZUMAB**_ if _**HER2/neu_** and node+
do not use **_Aromatase Inhibitors_** in premenopausal women.
36
CA 125 / how useful is it?
* 80% with ovarian cancer
**_also with +ve_**
* Cervix, breast, Endometrial, Lung, Colon
This is more like a monitoring factor while getting treatment.
37
1. Cancer Incidence
2. Cancer Death
* Male
* Female
Cancer Incidence
* Males : **Prostate** \> Lung \> Colorectal
* Females : **Breast** \> Lung \> Colorectal
Cancer Death
* Males : **Lung** \> Prostate \> Colorectal
* Females : **Lung** \> Breast \> Colorectal
38
Carcinoma in Situ -Lobar
**_Lobular_**
* Nonpalpable with no reliable mammographic changes
* Marker for cancer development in either breast
* Frequently is **multifocal and bilateral**
**_Treatment**_ varies from _**observation only to tamoxifen_** to bilateral mastectomy
39
Carcinoma In Situ - Ductal
**Ductal (DCIS)**
* Benign
* More common than lobular
* See **_microcalcifications_** on mammogram
* **_High-grade has highe_**r risk of evolving into invasive disease
**_Treatment_** is mastectomy vs. breast conservation surgery +/- XRT followed by tamoxifen
40
Starry Skies Burkitts
41
Cervical Cancer
• Treatment
- CIN I:
- CIN II:
- CIN III:
- CIN I: observation vs. cryotherapy
- CIN II: ablation or excision
- CIN III: ablation or excision
42
Causes of EPO resistance
* Iron def
* B12 or folate def
* Inadequate HD
* Infection
* Extensive hyperparathyroid bone dz
* Occult malignancy
* Medication (ACEi ARB)
43
Cervical cancer screening
1. HIV/SLE/Transplant
2. AGE\< 21
3. 21-29
4. 30-65
5. \>65
1. onset of sex Q6M X2 then yearly
2. No screening
3. Cytology Q3Y
4. Cytology Q3Y or Cytology with HPV Q5Y
5. No screening if -ve (**_3 paps or 2 HPV_**)
44
CLL
• Commonly associated with second malignancies
* Lung cancer
* Head and neck cancer
45
Chronic Lymphocytic Leukemia
Tx:
Indication
**_Treatment:_**
* Chlorambucil
* Fludarabine
* Combination chemotherapy
* Alemtuzumab
* Rituximab/ofatumumab
**_Indications_** for treatment include
* Symptomatic disease
* Rapid doubling of the WBC count \< 1 yr
* Anemia
* Thrombocytopenia
46
Chronic Lymphocytic Leukemia
Staging
RAI staging system Survival:
* 0 Lymphocytosis \>10yrs
* I. Lymphadenopathy 9 yrs
* II Splenomegaly 6-7 yrs
* III Anemia 2-3 yrs
* IV Thrombocytopenia 2-3 yrs
47
Clues on peripheral smear:
- Blasts =
- Auer rods =
- Mature cells =
- Mature lymphocytes =
- Maturing myeloid cells =
- Blasts = acute leukemia
- Auer rods = myeloid blasts
- Mature cells = chronic leukemia
- Mature lymphocytes = CLL
- Maturing myeloid cells = CML
48
Chronic Lymphocytic Leukemia [ALWAYS on Boards]
Work up
* A bone marrow aspirate and biopsy
* Flow cytometry on peripheral blood
* Cytogenetics on bone marrow
* Flow cytometry, which is usually diagnostic
* **_Typically a B-cell disorder_** with an abnormal pattern
* **_CD19, CD20, CD23_** Positive (BCell usually +ve for CD 19/20/23)
* CD5 Positive as well
* Light chain restricted
49
Chronic Myeloid Leukemia
* Typical laboratory presentation
* High WBC count / "**_Myelocyte spike_**"
* Eosinophilia and basophilia
Labs :
* Low **_LAP_** (leukocyte alkaline phosphatase) (not done now a days)
* Marrow that is hypercellular
* Characteristic chromosomal abnormality / **_t(9,22)_**
Few side effects primarily fluid retention
* Imatinib (Gleevak)
* Nilotinib
* Dasatinib
Bone marrow transplantation
* Still therapy of choice for young patients with a matched available donor / Only curative therapy known to date
* Best when performed early in the disease
50
CML Accelerated phase:
* Increasingly difficult to control counts
* Increasing eosinophilia and basophilia
* Accumulation of additional cytogenetic abnormalities
* Treatment becomes more difficult
CML can becom AML or ALL
51
CML Chronic phase:
* Usually relatively asymptomatic
* Easily controlled counts
* **Disease duration**
* 2 years untreated
* 3-4 years with hydroxyurea
* 4-5 years with interferon
* Unknown with imatinib \*\*\*\* tyrosin kinase inhibitors
52
Colon Cancer / staging / treatment / post survalance
* All men and women, beginning at age 50 years, should undergo screening colonoscopy.
* STAGE I (T1-2 N0) STAGE II (T3-4 N0) **Stage III (N1 N2) no M --- VI M+ve**
* **_FOLFOX chemotherapy_**
* All patients with _stage III colon cancer, regardless of age, should receive postoperative adjuvant chemotherapy unless contraindicated_ because of medical or psychiatric disorders.
* Patients with rectal cancers that are not full-thickness lesions and do not have obvious lymph node involvement typically are managed with primary _surgical resection_.
* Postoperative surveillance for _stage III colon cancer includes physical examination and carcinoembryonic antigen monitoring every 3 to 6 months_; chest/abdomen/pelvic CT scanning annually for 3 to 5 years; and colonoscopy 1 year after resection and then repeated at 3- to 5-year intervals.
53
* Cushing Syndrome
* Diagnosis is made by:
* Treatment options include:
**_Cushing Syndrome_**
* Small cell carcinoma, carcinoid tumors, and in association with MEN I syndrome / Ectopic ACTH
* Seen with hypokalemic alkalosis
* weakness, hypertension, & hyperglycemia
* buffalo hump, moon faces, hyperpigmentation, hirsutism
**_Diagnosis is made by:_**
* Elevated ACTH
* Cortisol levels that do not suppress with dexamethasone
**_Treatment options include:_**
* Treatment of the primary tumor
* Inhibitors of steroid synthesis such as **_ketoconazole or aminoglutethimide_**
54
Dermatomyositis
Dz
Dx
Tx
* **_Visceral adenocarcinomas_** such as stomach, breast, lung, and ovary assiciated dz
* Polymyositis associated with skin changes
* Proximal painless muscle weakness
* Violaceous rash on exposed parts, especially the eyelids
* Dysphagia is common
**_Diagnosis_**:
* Elevated CPK, aldolase, LDH, and ESR
* Abnormal EMG
* Muscle bx necrosis of muscle fibers and an inflammatory reaction
**_Treatments include_:**
* Steroids
* Treatment of the underlying malignancy
55
DIC Smear
* P prolonged
* PTT prolonged
* PLT Low
* Fibrinogen Low
* D-Dimer +ve
* Protamine +ve
On Smear
* Schistocytes
* Thrombocytopenia
* Retic ount Eelevation
56
Diagnose polycythemia vera
Identification of the JAK2 V617F mutation in patients with a hemoglobin level greater than 18.5 g/dL (185 g/L) in men or greater than 16.5 g/dL (165 g/L) in women, with concomitant leukocytosis, thrombocytosis, and hepatosplenomegaly, is diagnostic of polycythemia vera.
57
DIC tx
vWD tx
acquired hemophilia Tx
DIC tx resh **_frozen plasma and cryoprecipitate_**
vWD tx **_Desmopressin /vW factor if avaialbe _**
acquired hemophilia Tx **_Recombinant activated factor VIIa_**
58
DIC treatment
* Treat underlying disorder
* Support with appropriate products
* Cryoprecipitate
* FFP
* Platelets
* pRBCs
* Heparin therapy - rarely needed
59
**Eaton-Lambert Syndrome**
* Small cell lung cancer
* Variant of myasthenia gravis
**_Initial symptom:_** Weakness of the proximal muscles
* **_Autoimmune_** in nature, secondary to autoantibodies that result in **impaired release of acetylcholine **
**_Diagnosis_**
* abnormal EMG with **improvement in strength upon repetitive stimulation**
* EMG is usually unaffected by addition of edrophonium chloride
**_Treatment:_**
* **Treatment of malignancy**
* Guanidine
* Plasma exchange
* **IVIG**
* **Immunosuppression**
60
Elevated PT and PTT
**1:1 inhibitor mixing**
## Footnote
if corrects look for factor def
if does not correct late for **_coagulation factor inhibitors_**
WET - Warfarin Extrinsic PT (27 910) Vit K
HITT - Heparin Intrinsic PTT 89
61
Diabetic amyotrophy
Asymmetric focal lower Ext pain associated with muscle atrophy, areflexia, atunomic dysfunction and unintentional weight loss
62
Essential Thrombocytosis - Dx Need rule out:
* Thrombocytosis sustained over **_6 months_** that is unexplained
* May be associated with splenomegaly but not usually massive
* No clear diagnostic tests exist
* Although in about **_50% JAK-2_** is abnormal
* **_R/O_** IDA / Malignancy / Ifection / Malignancy
Treatment options include:
* Observation
* Hydroxyurea and busulfan
* Interferon-a
* Anagrelide
63
EXTRINSIC Factors
7
VII
WET Warfarin Extrinsic PT
64
Factor VIII Inhibitors
* **_IgG antibodies_**
* Detected when the 1:1 mix does not correct
* **_Treat with activated IX**_ product, porcine VIII, Factor Vila (NovoSeven®), or _**immunosuppression_**
65
first-line agent in constipation-predominant irritable bowel syndrome
Lubiprostone is not a first-line agent in constipation-predominant irritable bowel syndrome, but it is appropriate for patients whose symptoms persist despite the use of fiber and standard laxatives.
66
Treat a patient with advanced symptomatic follicular lymphoma.
**Rituximab** (2 yrs), combination chemotherapy, and **prednisone followed by rituximab** maintenance therapy
67
Germ cell
Treatment
* Fewer than 5%
* Usually large tumors
* May secrete **_aFP or bHCG_**
* Presents with early-stage disease
_**Treated** with surgery, followed by _
* chemotherapy with agents that include
* **_BEP_** (bleomycin, etoposide, and cisplatin)
68
Small intestinal bacterial overgrowth
Should be considered in patients presenting with diarrhea, bloating, or weight loss; **vitamin B12 deficiency or an elevated serum folate level** can be laboratory clues to the diagnosis.
69
Gleason Score
• Gives a grade to both the predominant and secondary growth pattern, and adds the scores
• Total will be 2-10, with higher grade correlated with higher incidence of spread and mortality
**_• 7 or \> Gleason score is high-risk disease_**
70
Hairy Cell Leukemia
(B-Cell)
- B cell in phenotype Usual presentation is:
* TRIAD
* Pancytopenia
* Large splenomegaly
* Inaspirable bone marrow
* Bone marrow with hypercellularity and increased reticulin fibrosis "dry tap"
* **_TRAP_** stain positivity
* Tartrate-resistant acid phosphatase stain
A single cycle of parenteral **_cladribine_** is curative in more than 80% of patients with hairy cell leukemia.
Also : “dry tap” and a biopsy shows diffuse infiltration with small lymphocytes with hair-like projections expressing CD20 but not CD5.
71
Hb AS
* Unable to **_fully concentrate urine_**
* Sickle Cell Trait
72
Hemoglobin SS
* Pain crisis
* Nonhealing leg ulcers
* AVN
* Aplastic crisis from parvovirus B19
* Functional asplenia
* Develop microinfarcts of organs, such as brain — CVA
* Develop priapism and also retinal detachment
73
Hemophilia A Tx:
**_Factor VIII Def_**
* Synthesized in the liver
* Need 25% activity for normal hemostasis
* Severe disease \< 1% activity
* Moderate disease 1-5% activity
* Mild disease \> 5% activity
* **_Tx:_**
* Mild **_DAAVP_** /
* for mild or moderate \> [**_Factore VIII_** or Cryopercipitate
74
Hemophilia B Tx:
**_Factor IX deficiency_** / Synthesized in the liver
* Requires vitamin K as a cofactor for modification
**_Tx:_**
* FFP
* Factor IX concentrates — monoclonal and recombinant are available
* Twice the volume of distribution to consider when calculating replacement doses
75
Hereditary Hemochromatosis
Dx About the Dz
* **_Transferrin saturation_** (***best test for Dx***) \> **_45% women and \> 50% men_**
* **_Ferritin_** \> 1,000 (in women it will take longer to accumulate 1000 due to bleed)
* **_Liver biopsy_** (Gold standard)
About the Dz
* Autosomal and recessive inheritance
* Frequent in Caucasians
* Mutation in C282Y or H63D genes
* Modulates absorption of iron via HFE protein (increase absorption)
76
bladder cancer is treated: (High-risk, early-stage )
High-risk, **_early-stage bladder cancer is treated_** with intravesicular medication, typically bacillus Calmette-Guérin immunotherapy.
77
HIT Tx:
* Discontinuation of heparin or LMWH
* Substitution of lepirudin(Renal) or argatroban (Liver)
* Initiate warfarin only after platelet count has recovered and anticoagulation with alternate agent is established
78
Hodgkin Lymphoma
Complications post treatment
* Hypothyroidism (post radiation)
* Infertility (MOPP)
* Secondary malignancy, including
* MDS/AML
* Solid tumors such as breast and lung
* Must screen early
79
Hodgkin Lymphoma Dx
* Diagnosis made by finding **_Reed-Sternberg cells_**
* WHO defines classical Hodgkin's and NLPHL
80
Hodgkin Lymphoma
**_SX:_**
* Bimodal age distribution (young and old)
* Often see the "B" symptoms
* Fever
* Night sweats
* Weight loss
* Pruritus is common
* **_Unusual complaint of pain with alcohol ingestion_**
81
Hodgkin Lymphoma
**_Tx_**
* Treatment involves either:
* Combination chemotherapy
* ABVD or MOPP
* Radiation therapy
* Combined modality therapy with both
82
Hypercalcemia :
Tx: Medications:
**_Steroids_**
* Primarily **_MM_**, also lymphoma and occa. in breast cancer / tumor is responsive
* Blocks Osteoclast activating factors + May also increase calcium excretion
**_Mithramycin_**
* Reduces the # and activity of osteoclasts - given in IV form
* A/E thrombocytopenia, liver tox. & coagulopathy
**_Calcitonin (quick)_**
* Inhibits bone reabsorption by binding
to osteoclasts
* Tachyphylaxis
**_Bisphosphonates (Mainstay of tx)_**
* Concentrated in high turnover area inhibits osteoclasts
* Pamidronate has proven efficacy in all types of high Ca w low skeletal events
in both breast cancer & MM
* Zoledronic acid also approved for
malignant hypercalcemia
* Toxicity is primarily renal insufficiency
83
Hypercalcemia
* Occurs frequently with
* Symptoms resulting from hypercalcemia:
* Life expectancy
* Occurs frequently with
* squamous cell carcinomas,
* breast cancer
* renal cell carcinoma
* Multiple myeloma
* Symptoms resulting from hypercalcemia:
* Fatigue, constipation, Polyuria, polydipsia
* Mental status changes, Renal dysfunction
* Shortened QT, PR prolongation, Brady
* hyporeflexia
* Life expectancy \< 6 months
84
Hypercoagulable states (Genetics)
* Antithrombin III Deficiency
* Protein C Deficiency
* Protein S Deficiency
* Protein C/S Deficiencies
* Mutation in Factor V laden
* Antiphospholipid Antibody Syndrome
85
Hypersegmented
6 lobes or many 5 lobes
86
CSF Analysis
* Normal
* Bacterial meningitis
* TB meningitis
* Viral Meningitis
* Guilain Barre
* WBC / Gluc / Protien
* Normal 0-5 / 45-80 / 18-58
* Bacterial m.\>1000/ 400
* TB m. 5-1000 / \< 10 / \>400
* Viral M. 100-1000 / 45-80 /
* Guilain B. 0-5 / 45-80 /45-1000
87
* Imatinib
* Induction chemotherapy
* Leukapheresis
* Rituximab
* Imatinib : BCR-ABL inhibitor used for the treatment of chronic myeloid leukemia. It is used to treat Philadelphia chromosome-positive ALL
* Induction chemotherapy for ALL : aunorubicin, vincristine, L-asparaginase, and prednisone.
* Leukapheresis : hyperleukocytosis,
* Rituximab: Leukemia / RA / MM
88
INR toxicity
Any with bleed Hold warfarin / IV K 10 mg, FFP, Recombinant factor VIIa or prothrombin comples concentrate.
\< 5 no bleed whold warfarin
\< 10 hold warfarin give oral K if think bleed is possible
\> 10 hold warfarin and give K2.5-5 mg oral K
89
detection of insulinomas
**Endoscopic ultrasound** has an approximately 90% detection rate for insulinomas.
90
INTRINSIC factors
8 9 11 12 -
XII XI IX VIII
HITT
91
Manage a patient with early-stage invasive ductal carcinoma
* Breast conservation therapy, which consists of **_excision of the primary tumor and radiation therapy,_** is equivalent to mastectomy in long-term survival.
* Mastectomy is indicated for patients who are not eligible for breast conservation (those in whom complete excision is not technically possible with lumpectomy alone or those in whom radiation is contraindicated).
92
Iron deficiney Anemia / What to look for?
* Microcytosis and hypochromia
* Elevated RDW
* Decreased reticulocyte count
* Elevated platelet count
* Remember **_Soluble Transferin receptor_** is elevated as well
93
Iron deficiney Anemia Gold Standard
* Iron Low
* TIBC High
* % sat Low
* Ferritin Low
* Bone Bx most definite Dx
94
ITP
* Viral illnesses such as _HCV, HIV_
* No diagnostic test
* Primary is a diagnosis of exclusion
* Rule **_out SLE_**
* Evaluate drug history
In children self limite in adults chronic
95
ITP associated with?
SLE
96
ITP Tx:
* Platelets \> 30,000 — observation
* Platelets \< 30,000 — treatment
* Steroids / IVIG / Splenectomy (will fix 80%)
* Optional therapies for patients failing steroids and *_splenectomy_*
* ***_Rituximab_*** \*\*\* /
* Thrombopoietin **_TPO receptor agonists_** \*\*\*
97
JAK 2
polycythemia vera or essential thrombocythemi
98
**_Lenalidomide_** in combination with high-dose dexamethasone.
Associated with an increased risk for venous thromboembolism in patients with multiple myeloma.
99
1:1 Mixing
Would fully resolve if it was Factor def
**Inhibitor / aquired inhibitors** whould take longer then normal but less then no-mix
**Factor XI def c**ould lead to prolonged mixing study
100
macroovalucyt-b12folate
101
Leukostatsis
Extremely hight WBC ~100000
I**_nduction of chemo_** # 1 if not pregnent
**_Hydroxyurea_** # 2 if not pregnent and not symptomatic
**_Leukapheresis_** is # 2 if pregnenet
102
microscopic colitis
* Histologic changes limited to the colon, unless it occurs in the setting of celiac disease.
* Colonic disease, features of fat malabsorption and vitamin deficiencies should not be seen.
103
Manage short-bowel syndrome
patient should receive a proton pump inhibitor (PPI) such as omeprazole. In patients who have undergone massive resection of the small intestine and are left with short-bowel syndrome, there is a tremendous surge of gastric acid in the postoperative period. The increased acid can inactivate pancreatic lipase, leading to significant diarrhea and possible ulceration in the remaining bowel. Therefore, all patients who have undergone significant bowel resection should receive acid suppression therapy in the postoperative period with a PPI.
Although increasing the loperamide may help with diarrhea control, it will not target the underlying pathophysiology of the increased acid production and will not prevent small-bowel ulceration.
104
Manage stage III colon cancer.
FOLFOX
An adjuvant chemotherapy regimen of 5-fluorouracil, leucovorin, and oxaliplatin (**_FOLFOX_**) has been shown to improve disease-free survival in patients with stage III colon cancer.
105
mantle cell lymphoma
Bone marrow biopsy reveals diffuse infiltration with small monoclonal lymphoid cells. Immunohistochemistry discloses overexpression of cyclin D1, and cytogenetic analysis shows a **_t(11;14) translocation._**
106
Manage a patient with a history of infective endocarditis before a dental procedure.
The indications for infective endocarditis antimicrobial prophylaxis for patients who will undergo a dental procedure involving manipulation of gingival tissue or the periapical region of teeth or perforation of the oral mucosa are
(1) the presence of a prosthetic cardiac valve
(2) a history of infective endocarditis
(3) unrepaired cyanotic congenital heart disease
(4) congenital heart disease repair with prosthetic material or device for the first 6 months after intervention
(5) presence of palliative shunts and conduits
(6) cardiac valvulopathy in cardiac transplant recipients.
107
A. 1:1 mix of patient and normal plasma
B. Bleeding time/PFA 100
C. Antiplatelet antibodies
D. Factor VII level
pick one for each : vWD / Factor deficiency / Hemophillia
A. Factor deficiency with 1:1 mix currects Inhibitors do not correct with 1:1 mixing [first test for abnormal PT PTT]
B. vWD
C.
D. Hemophillia
108
MDS (myelodysplastic syndrome)
* asymptomatic
* cytopenia (megaloblastic anemia with lower reticulocyte response)
* thrombocytopenia and neutropenia later
* hypercellular marro with single or multi lineage dysplasia
* Rule out folate / B12 (hyperlobulated neutorphil)
109
Manage early-stage melanoma / 1 mm is the key / sentinal LN resection if bigger then this
Tumors thicker than 1 mm require a **_2-cm margin of resection_**, and tumors with clinically positive nodes require lymph node dissection.
110
Metastic bone dz
1. Alendronate
2. Denosumab
3. zoledronic acid
4. pamidronate
* 1. Alendronate
* 2. Denosumab
* 3. zoledronic acid
* 4. pamidronate
1. not for metastic bone dz
2. good **but not for MM**
3. all good
4. all good
111
MGUS therapy
MGUS
• No therapy is indicated, but these patients should be followed at least yearly for progression of their
disease
112
MGUS
* **_\< 10 % monoclonal plasma c_**ellsin BM
* **_non IgM_** - IgG, IgD or IgA / can progress to MM / Less frequent AL amyloid
* **_IgM Can progress to Waldenstrom_** macrogammagolbulinemia
* less frequent
113
MM
* CRAB
Calcium / Renal fail / Anemia / Bone lytic
* non IgM monoclonal \> 3 g/dL
* \> 10 plasma cells on BM
* Elevated B2 microglogulin
114
MM
* 95% will have an abnormal protein on SPEP or UPEP
* The **_M spike**_ is most commonly _**IgG followed by IgA_**, light chain disease, and IgD \< 5% will be non-secretory with no evidence of protein secretion
115
**_MM _**Clues include:
* A low anion gap
* Rouleaux on peripheral smear (when protien levels are high makes the RBC sticky)
* An elevated globulin fraction (Total Protien - Albumin ~ 3.5)
* Remember that the bone lesions seen with myeloma are purely lytic lesions
* They should be assessed with metastatic bone survey, NOT bone scan
TX
- Melphalan and prednisone
- Combined chemotherapy regimens
- Thalidomide/lenalidomide
116
Myelodysplastic Syndromes (MDS)
Dx:
* bone marrow evaluation revealing **_dysplastic maturation of 1 to 3 cell lines_**
* hypercellular bone marrow with evidence of dysplasia Prognosis depends on _**Percentage of blasts** _
* Cytogenetics Smear **Dimorphic RBC / Macrocytic RBC** Decreased reticulocyte count
* Ringed **_sideroblasts_** \*\*\*\* iron deposits
117
Myelodysplastic Syndromes (MDS) Tx
* Supportive care
* B6, B12, and folate
* Growth factors
* epo
* neupogen
* Chemotherapy
* Bone marrow transplant
118
Idiopathic Myelofibrosis
Smear?
* Older patients
* Clonal disorder with cytopenias
* Marrow fibrosis
* **_Extramedullary hematopoiesis**_ (make blood in spleen and liver / _**massive spleenomegaly_**)
* Smear:
* Tear drop RBC / (nucleated )nRBCs / Left-shifted WBC series
119
Myelofibrosis /Treatment options include
* Supportive care
* Growth factors PRN
* Hydroxyurea or busulfan / to help to shrink the splee
* Splenectomy / may the only source of thier RBCs production be careful
* Bone marrow transplant
120
Myeloproliferative Disorders
* Older patient
* Clonal disorders
* Hypercellular BM
* Splenomegaly
* Polycythemia vera
* Essential thrombocytosis
* Myelofibrosis
* Chronic myeloid leukemia
121
1. Dementia with lewy bodies
2. Idiopathic parkinsons dz
3. multiple system atrophy
4. progressive supranuclear palsy
5. sporadic spongiform encephalopathy
Dementia with lewy bodies
* Dysautonomics not seen as much as MSA
* rapidly progressive dementia / visual hallucination fluctuating cognition, REM sleep d/o, parkinsonism
Idiopathic parkinsons dz
* Slowly progressed / REM behaviour d/o, dyautonomia \> 10 yrs, resting tremor /rigidity etc.
multiple system atrophy
* xsynucleonopathy, radip progression, prominent dysautonomia (urinary incontinenec, ortho hypotension), ataxia w falls, parkinsonism
progressive supranuclear palsy
* rapid progression, impared vertical saccades, parkinsonism, absent of gait problems or tremors
sporadic spongiform encephalopathy
* Creutzfeldt Jakob Dz / repid progression, slurred speech, ataxia, myoclonus, hallucinations
122
Neuropsych symptoms as agitation and aggression hallucination in patients with dementia ! what to do.
Behavioral and eviromental therapy is the key
Cholinesterase inhibitors : improves cognitionin dementia not behaviour
Low dose Lorazepam: good for Alzheimer's aggitation only need to be brief
**_Atypical neuroleptics_** - not approved for hallucinations in dementia
123
Non hodgkin lymphoma
* Relapse chances
* Secondary Malignancies?
* Other Dz associated with post tx?
* After 5 yrs of screening the relapse chance is very low.
* Breast mammogram **_10 yrs post RTx_**/ lung / skin exam yearly
* Cariology referal needed / primary prevention needed (control HTN, smoking, obese, LDL)
* Endocrine (thryoid exam function / reproductive endocrinology
* Neuro psych - (depression PTSD)
124
Non-Hodgkin Lymphoma
* Low Grade
* Indolent course
* Older patients
* Fewer "B" symptoms
* Higher stage at presentation
* **_Predominantly follicular lymphomas_**
* Sensitive to chemotherapy but not curablelesions
* Long median survival: 7-10 years
* May upgrade to more aggressive disease
125
**NHL:
Treatment strategies:**
* *_• Low-grade lesions_**
- "Watch and wait" with treatment reserved for symptomatic disease
- Treat until symptoms resolved, then observation again
- Treatment should be more aggressive if younger patient
* *_• Intermediate and high-grade lesions_**
- Treat at time of diagnosis
- Use combination chemotherapy +/- radiation therapy
- Standard of care is CHOP plus monoclonal antibody therapy with rituximab
126
Non small cell lung cancer
Treatment
Treatment options
Cytotoxics (platinum compounts)
Targeted agents(EGFR inhibitors, ALK fusion inhibitors, monoclonal antibodies)
127
Non-Hodgkin Lymphoma Burkitt Lymphoma = L3 ALL
**_Endemic Epidemic_**
African variety U.S.
Jaw mass Abdominal mass
EBV+++ EBV+/-
* Very rapid growth pattern/aggressive
* Looks like "starry sky" under low power
* **Associated with a t(8,14)**
* Involves c-myc proto-oncogene
* Moves to immunoglobulin gene loci
* Involves CNS and marrow frequently
128
Ovarian Cancer
• Staging should include:
* CA-125 level
* Chest x-ray
* Abdominal CT scan
* Abdominal ultrasound
* +/- Upper Gl series and barium enema
129
Ovarian Cancer
Risk factor
Screening
Family history
* Most significant risk factor
* Up to 10% may be genetically based
* Breast-ovarian syndrome
* BRCA1 — 40% risk
* BRCA2— 10-20% risk
* Lynch II syndrome
* 12% risk
Screening at this time is restricted to **BRCA1 or BRCA2**
* Pelvic exam
* Transvaginal ultrasound
* CA-125
* NOT REALLY EFFECTIVE
130
Ovarian Cancer / Treatment options
**_Primary surgical resection_**
* The amount of remaining disease has a direct correlation to long-term survival
**_Chemotherapy_**
* Combinations of **_paclitaxel and cisplatin_** are standard of care
**_Radiation therapy_**
* Not used often (**_Cervical cancer Rad is useful_**)
131
Ovarian cancer relapse / how to follow up with them.
* Ovarian cancer relapse rates are high **_(\>70%) in patients with stage III and IV disease,_** and treatment for relapse can prolong survival; therefore, once-yearly follow-up is not frequent enough.
* History, physical examination, pelvic examination, and **_CA-125 measurement every 4 months_**
132
Pain management
Mild
Moderate
Severe
Mild OTC stuff
Moderate : Codein / tramadol / hydrocodone
Sever : Morphine / hydromorphine
could also consider patch /oxycodone with short acitng morphins
133
Paroxysmal Nocturnal Hemoglobinuria / Dx
Gold is Flow cytometry
missing **_DAF CD 55 & HRF CD59_**
* What to see in blood :
* low hemoglobin / haptoglobin
* raised LDH / retic / bili
* Associated with risk for thrombosis (abd thrombos / Budd–Chiari in patients) / blood in urine
* RBCs more susceptible to complement degradation secondary to the absence of Decay accelerating factor (DAF)
* Homologous restriction factor (HRF)
134
Pelger-HuetCells-Myelodysplasia
Pelger-HuetCells-Myelodysplasia
135
Pernicious anemia
Autoimmune in nature with associated
* antiparietal cell antibodies
* or intrinsic factor antibodies
Look for Antibodies for diagnosis but do not have to be +ve
136
Pilocarpin
cevimeline
**target M3 receptors** to stimulate saliva production good for patients that had chemo
137
myeloma-plasmacells
138
Polycythemia Vera
* JAK 2
* Low erythropoietin
* low risk for thrombosis can give ASA
* phlebotomy for target hematocrit of 45-50%
* Systemic treatemnt Antihistamine for pruritus
139
Polycythemia Vera dx
JAK-2 genetic / spenomegaly / blood cloths / hyperviscocity / Erythromelagia
Diagnostic criteria: (modified from the PVSG)
* Increased RBC mass
* Normal PaO2
* Splenomegaly
* Low serum erythropoietin levels
140
Polycythemia Vera Symptoms
* Plethora / Splenomegaly / Thrombosis
* Hyperviscosity / Gout /
* Erythromelalgia
* Erythema and pain of digits
141
Polycythemia Vera tx
* _Phlebotomy_ — easy way to control red blood cell count
* Drugs — used primarily to help control platelet count or if phlebotomy not feasible / _hydoxyurea_
* _Low-dose ASA_ — if not contraindication
142
Tomoxifen & Aromatase inhibitors prolong use AE
Prolonged tamoxifen use include:
* Increased risk of **_endometrial cancer_**
* **_Risk of thrombosis DVT/PE_**
* Cataracts
Aromatase inhibitors now also proven for prevention
* **_Osteoporosis_**
* Arthralgias
* No thrombosis or malignancy risk
143
Porphyria Cutanea Tarda
Associated with:
HBC most common / HIV / ETOH / Estrogen use / Smoking
Clinical presentation:
Skin blister / bullae, scarying, hypo/hyperpigmentation on sun exposed area
Elevated urine uroporphyrin
144
Prognositic Factors:
* ER/PR Hormone receptor status?
* HER2/neu status?
**_Hormone receptor status_**
* **If ER/PR receptor-negative**, then expect a **higher _incidence of recurrence_**
**_HER2/neu status_**
* **Overexpression of HER2/neu** denotes a **_more aggressive tumor_**
145
Prostate Cancer
* Front-line therapy is generally hormonal manipulation
* Remember that the prostate is androgendependent, and most cancers maintain some degree of sensitivity to androgens.
* Hormonal manipulation may be accomplished using:
- Anti-androgens
- LHRH agonists
- Orchiectomy
146
Prostate Cancer
Treatment?
Localized disease (Stage I and II)
* Goal is curative therapy
* Radical prostatectomy vs. radiotherapy is the usual dilemma if life expectancy \> 10 years
* Different side-effect profiles
* Selection is patient-dependent: If patient is elderly, then observation is a viable option
Stage III disease, which penetrates the capsule
* into surrounding structures, is best treated with radiation therapy alone
Stage IV disease with spread to nodes or distant
* sites is not a curable illness
* Common sites of spread include:
* Skeletal system / lungs / Liver
147
Prostrate cancer
**_Radiation therapy_** appropriate for patients at low or average risk
**_Radical prostatectomy_** is recommended for younger patients with organ-confined disease and a life expectancy greater than 10 years. This patient's age and underlying medical problems make him a poor candidate for aggressive surgical intervention.
148
RBC "Clues"
• Cigar/Pencil Shapes
• Macroovalocytes
• Spherocytes
• Schistocytes
RBC "Clues"
* Cigar/Pencil Shapes = iron def anemia
* Macroovalocytes = B12 Folate
* Spherocytes = autoimmune hemolytic anemia
* Schistocytes = DIC / TTP / HUS
149
RBC "Clues"
• Bite Cells
• Burr Cells
• Target Cells
• Teardrop Cells
RBC "Clues"
* Bite Cells = G6PD def
* Burr Cells = Renal Dz / Uremia
* Spur Cells = Liver Dz
* Target Cells = Liver Dz , Hemoglubin C Dz / cholestrol dz
* Teardrop Cells =Myelofibrosis
150
Risedronate / zoledronic acid
When what why treatment?
**_Risedronate_** is for paget's dz / Osteoporosis
**_Zoledronic_** acid is approved for bony mets of solid cancers
151
Prostate Cancer
Screening recommendation from the ACS (2010):
* Discuss with physician to make informed decision after age 50 or after age 45 for high-risk individuals
* **_USPSTF_**: Insufficient evidence prior to 75 and recommends against for \> 75 years of age
152
Breast Cancer
What they agree on (all of them)
Guidelines share
* **_No clear recommendation_** for BSE (self exam)
* **_No clear recommendation_** for CBE (Clinical Exam)
* MMG from 50-74 either **yearly or every 2 years (age 40-49 discuss with patient)**
153
Cervical Cancer
* Pap smear classified using the Bethesda system
* What to do?
* WNL
* Benign cellular changes
* Atypical squamous cells/glandular cells of undetermined significance (ASCUS/ASGUS)
* LGSIL (**low-grade squamous intraepithelial lesion**)
* HGSIL (**high-grade squamous intraepithelial lesion**)
* Cancer
**_What to do?_**
**ASCUS**
* repeat in 3-6 mos
* Abnormal repeat Pap needs colposcopy
**ASGUS**
* Colposcopy
**LGSIL (CIN I) / HGSIL** **(CIN II III)**
* Colposcopy and Biopsy
154
Cervical Cancer — Screening
* Begin **_no later than 21_**
* Age **_21-30 do every 1-2 year_**s
* After **_age 30 do every 2-3 year_**s
* Stop after hysterectomy for **_benign disease**_ or _**age 65-70_**
155
Seminomatous or nonseminomatous testicular tumors.
An elevated serum **_α-fetoprotein level a**_lways indicates that a testicular tumor has a _**nonseminomatous_** component, whereas elevated β-human chorionic gonadotropin may be present in seminomatous or nonseminomatous testicular tumors.
156
SIADH
**_SIADH_**
* Small cell lung cancer
* Medications: Cyclophosphamide (Cytoxan® or Neosar®)
* Results hyponatremia
* Mental status changes, nausea, anorexia, and weakness
* Coma, seizures
**_Treatment options include:_**
* Fluid restriction — works best in mild cases
* IV saline (0.9-3%) plus furosemide
* **_Conivaptan_** for acute treatment
* **_Demeclocycline_** for chronic treatment
* Treat the _underlying malignancy_
157
Soluble transferrin receptor
If high its Iron Def
158
Spherocytes
159
Suspected ITP
**\> 30000 no bleed / observe**
\< 30000 with bleed Then
First line
Second line
third line
1. Treat with **Steriods or IVIG**
2. **Slenectomy** or rituximab
3. thrombopoiesis stimulating agent
160
Symptomatic myeloma dx:
* C**_lonal plasma cells \>10% on bone marrow biopsy_** or (in any quantity) in a biopsy from other tissues (plasmacytoma)
* A **_monoclonal protein_** (paraprotein) in either serum or urine (except in cases of true non-secretory myeloma)
* Evidence of end-organ damage felt related to the plasma cell disorder (related organ or tissue impairment, ROTI, commonly referred to by the acronym "CRAB"):
* Hyper**C**alcemia (corrected calcium \>2.75 mmol/L)
* **R**enal insufficiency attributable to myeloma
* **A**nemia (hemoglobin
* **B**one lesions (lytic lesions or osteoporosis with compression fractures)
161
testicular cancer
NonSeminoma
**_Non **_has all in _**ALL_**
AFP and B-hCG
Seminoma B-hCG only
162
Testicular Cancer
• **Diagnosis**
* Often an initial **_trial of antibiotics_** is given if epididymitis or orchitis is suspected
* **_Testicular ultrasound_** is indicated for a painless mass or painful one that does not resolve after antibiotics
* Ultrasound can distinguish between a **_solid mass and inflammatory changes_**
* Markers, including **_p-hCG, AFP, and LDH_**, are common
163
The best available therapy for chronic hepatitis C
peginterferon and ribavirin, with the addition of an NS3/4A protease inhibitor for genotype 1 hepatitis C virus.
164
abciximab / tirofiban and eptifibatide
GP IIb-IIIa inhibitors
pt can develop thrombocytopenia within a few hours starting the infusion. Recovery in 1-2 days can transfuse plt if low or bleeds
How is it defferent then HIT ?
HIT : usually needs 5-9 days post exposure to heparin / could happen earlier if was exposed within last 1-2 months (preexisting ab)
165
Manage the transfusion requirements in a patient with thrombocytopenia and intracranial hemorrhaging.
Platelet transfusion to maintain the platelet count at 100,000/µL (100 × 109/L) for the first few days after central nervous system bleeding or immediately prior to and after a planned central nervous system surgery is recommended.
166
Thrombotic Thrombocytopenic Purpura - The peripheral smear is helpful / similar with DIC
* **_Schistocytes_** (shearing RBCs / because microangiopathic)
* **_Thrombocytopenia_**
* Normal WBC series
* Shift cells
* nRBCs (BM compensation)
LABS: usually look for hemolysis
**LDH, Elevated Bili, ADAMTS13**
167
Blood transfusion
* Hb S negative
* Irradiated
* Phenotypically matched
* Washed
* Hb S negative decrease the risk of vasoocclusive complications
* Irradiated - Immunocompromised / graft
* Phenotypically matched / Leuoteri - Chronic infusions like Sickle cell
* Washed - IgA
168
TRASTUZUMAB
TRASTUZUMAB if HER2/neu +ve
Cardiomyopathy
169
Trastuzumab Common SE
Trastuzumab is associated with cardiac toxicity; patients who will receive adjuvant trastuzumab for 1 year require evaluation of the left ventricular ejection fraction before and during treatment.
170
Treat a patient with heparin-induced thrombocytopenia.
## Footnote
Because lepirudin is cleared through the kidneys so be cautouse with CKD
argatroban is cleared with liver
171
Treat a patient with kidney insufficiency and acute venous thromboembolism in the postoperative period
* Intravenous unfractionated heparin is the most appropriate treatment for deep venous thrombosis in patients who have undergone recent surgery and have chronic kidney disease.
172
Treat a patient with mildly symptomatic hereditary spherocytosis.
Patients with hereditary spherocytosis with mild asymptomatic disease should receive supportive care consisting of close clinical follow-up, maintenance of immunizations, and continued folic acid supplementation.
Patients with more severe disease leading to symptomatic **_anemia, growth retardation, skeletal changes, painful splenomegaly, or extramedullary hematopoietic tumors respond very well to splenectomy_**, with improvement in anemia, but it is not indicated in this patient.
173
Treat a patient with secondary iron overload from β-thalassemia major.
* Patients with secondary iron overload from thalassemia are best managed with iron **_chelators such as oral deferasirox._**
* Patients with _β-thalassemia major develop iron overload because of inappropriately increased iron absorption caused by ineffective erythropoiesis_ and from multiple transfusions.
174
Treat a patient with suspected advanced-stage testicular cancer.
* Inguinal orchiectomy followed by chemotherapy
* platinum, etoposide, and bleomycin (**_PEB_**)
* nonseminoma germ cell cancer had **_Alphf feta protien+_**
175
Treat a younger patient with high-risk acute myeloid leukemia
* **_Allogeneic hematopoietic stem cell transplantation_** results in an improvement in disease-free and overall survival in younger patients with high-risk acute myeloid leukemia compared with chemotherapy.
176
Treatment For IDA
**6 months** after their Hct normalizes you can stop
177
TTP Labs
* Elevated reticulocyte count
* LDH
* Total and indirect bilirubin
* **_ADAMTS13_** gene defect
178
Thrombotic Thrombocytopenic Purpura
Diagnostic Pentad:
FAT RN
* Fever
* Anemia Microangiopathic hemolytic
* Thrombocytopenia
* Renal dysfunction
* Neurological signs
179
TTP Tx
* Plasma therapy — preferably by **_plasma exchange if available_**
* Steroids
* **_Platelet transfusions are contraindicated unless there is a life-threatening bleed_**
180
vaccination in an immunosuppressed to AVOID
* Varicella vaccine
* yellow feverintranasal influenza
* measles-mumps-rubella
* bacillus Calmette-Guérin
* oral typhoid
181
Vit K def
- K-dependent factors
- Causes
* Vitamin K-dependent factors are
* II / VII / IX / X
* Protien C and S
* Vitamin K deficiency seen with
* Liver disease
* Poor absorption
* TPN
* Warfarin use
* Antibiotic use
* In acute cases give FFP otherwise Vit K po
182
von Willebrand disease and oral contraceptive
Patients with a personal and family history of mucocutaneous bleeding who have borderline-low levels of von Willebrand factor while taking oral contraceptive pills have possible von Willebrand disease.
183
von Willebrand Disease Labs
* _vWF:**Ag**_ — assay for the total vWF protein (levels)
* vWF: **_Ristocetin cofactor_** — assay for the ability of patient plasma to agglutinate normal platelets with the addition of ristocetin / RCoF (function)
* • **_RIPA_** — platelet aggregation in response to ristocetin
184
vWD - perioperative care
**_VWF concentrates_** are used perioperatively in severe VWD
DDAVP is effective in mild bleeding and in pt with mild to moderate Type 1 VWD
185
Waldenstrom Macroglobulinemia
Pathophysio
Presentation
Waldenstrom Macroglobulinemia
• Increased IgM levels
• More common in older men
• More consistent with a lymphoma with lymphadenopathy and organomegaly
- Purpura
- Neuropathy
- Hyperviscosity syndrome
186
Warm and Cold antibodies
Warm
* IgG
* lymphoproliferative and collagen vascular diseases
* **_Tx:_** Responds to immunosuppression / splenectomy
Cold
* IgM
* Hemolysis is via the complement pathway
* Associated with lymphoproliferative diseases or infection
* **_Tx:_** Steriods but does not really work
187
WBC "Clues"
• Dohle Bodies
• Pelger-Huet Cells
• Auer Rods
WBC "Clues"
• Dohle Bodies - infection / in nuetrophills gray bodies on the peripheral
• Pelger-Huet Cells MDS
• Auer Rods = AML
188
WBC "Clues"
• Smudge Cells
• Basophilia/Eosinophilia
• Hair-like Projections
• Hyperseg mentation
WBC "Clues"
• Smudge Cells = CLL
• Basophilia/Eosinophilia = Myeloprolifirative Dz (CML / ET / PV)
• Hair-like Projections = Hairy Cell
• Hyperseg mentation = B12/Folate Def
189
What causes hypocalcemia in plasma exchange procedures?
* Citrate, the anticoagulant used in most plasma exchange procedures, can lead to chelation of calcium and subsequent hypocalcemia.
190
* Differentiating between these:
* MGUS
* Smoldering MM
* Active MM
* **_MGUS_**
* **_\< 10% marrow_** Plasma Cellsand **\< 3 gm** M spike
* No end organ damage
* **_Smoldering MM_**
* 10% marrow Plasma Cells or \> 3 gm M spike
* No end organ damage
* **_Active MM_**
* 10% marrow Plasma Cells with an M spike and end-organ involvement
* Calcium, anemia, renal dysfunction,bone disease
191
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