Heme/Oncology Bonus Flashcards
(106 cards)
1
Q
What are the 3 diagnostic criteria for Multiple Myeloma
A
- > 20% plasma cells in bone marrow
- Monoclonal protein in serum or urine
- Evidence of end organ damage (CRAB)
2
Q
What is CRAB
A
- Elevated calcium
- Renal insufficiency
- Anemia
- lytic Bone lesions
3
Q
What are the bone problems in multiple myeloma
A
bone pain in back and thorax; and pathologic fractures
4
Q
What causes anemia in Multiple myeloma
A
plasma cells infiltrate the bone marrow, pushing out RBCs
5
Q
What causes infections in Multiple myeloma
A
recurrent infections result from impaired neutrophil function and deficiency of normal immunoglobulins (humoral deficiency)
6
Q
What causes nausea and vomiting in Multiple myeloma
A
Constipation and uremia
7
Q
What manifestation does hypercalcemia cause in Multiple myeloma
A
Delirium
8
Q
Multiple myeloma causes hyperviscosity…what are the results of that?
A
HYPOnatremia, Neurologic complications such as spinal cord or nerve root compression, blurred vision
9
Q
Patients with Multiple myeloma have purpura and epistaxis, why?
A
Thrombocytopenia
10
Q
General SandS of Multiple myeloma
A
Paresthesias, weight loss, generalized weakness.
11
Q
What type of anemia does Multiple myeloma cause? (think size)
A
Normochromic and normocytic, rouleaux formation
12
Q
What labs are elevated with Multiple myeloma
A
Elevated blood urea nitrogen, creatinine, uric acid and total protein
13
Q
Urine protein immunoelectrophoresis results in multiple myeloma
A
proteinuria from overproduction and secretion of free monoclonal kappa or lambda chains (Bence Jones protein)
14
Q
Serum protein immunoelectrophoresis results in multiple myeloma
A
monoclonal spike (M spike) on protein immunoelectrophoresis in approx. 75% of patients; decreased levels of normal immunoglobulins
15
Q
Which immunoglobulins increase with multiple myeloma
A
IgG (70%) and IgA (20%)
16
Q
What can the serum beta-2 macroglobulin test tell us about multiple myeloma
A
levels >8 mg/L indicate high tumor mass and aggressive disease
17
Q
What lab indicates a poor prognosis in multiple myeloma?
A
Elevated serum lactate dehydrogenase
18
Q
Abnormal chromosomes of Multiple myeloma found on FISH test
A
High-risk patients (<25% of patients at diagnosis) are those with any of the following: deletion 17p, translocation 4:14, translocation 14:16, deletion 13q, or cytogenetic hypodiploidy.
19
Q
TTP signs and symptoms
A
flu like symptoms, weakness, nausea, abdomen pain, vomiting, purpura (from thrombocytopenia), jaundice and pallor (from hemolysis), mucosal bleeding, fever, fluctuating level of consciousness (due to thromboses in brain), renal failure and neuro changes are end stage features
20
Q
What labs are elevated with TTP
A
Elevated BUN and creat, retic count, indirect bilirubin, lactate dehydrogenase, haptoglobin
21
Q
What labs are decreased with TTP?
A
- Decreased or absent activity of ADAMTS-13 and autoantibody inhibitor
- Hgb and Hct
- Thrombocytopenia, <50,000 platelets
22
Q
Urinalysis results for TTP?
A
Hematuria and proteinuria
23
Q
Fibrin levels in TTP
A
Normal fibrin level, Rule’s out DIC
24
Q
Treatment for acquired TTP
A
Immediate TPE for acquired TTP: plasma exchange, every day until LD and platelet count normal for 2 days. Done to replace ADAMTS13
25
Treatment for hereditary TTP
FFP infusion
26
Treatment for TTP if patient is allergic to plasma
Give factor VIII
27
Should you give platelets for TTP?
Platelet transfusions contraindicated unless thrombocytopenia severe and patient needing surgery or other invasive disease
28
Last resort treatment for TTP
Splenectomy
29
General signs and symptoms of CML
- Fatigue, night sweats, low grade fevers related to the hypermetabolic state caused by overproduction of white blood cells.
- Abdominal fullness related to splenomegaly.
30
Leukostasis symptoms with CML
Blurred vision, respiratory distress, or priapism. The WBC in these cases is usually greater than 100,000 but less than 500,000.
31
Signs of acceleration of CML
fever in the absence of infection, bone pain and splenomegaly.
32
WBC count for CML
elevated WBC, median WBC is 150,000 at diagnosis
33
Platelet count with CML
normal or elevated
34
What does bone marrow show in CML
- The bone marrow is hypercellular, with left-shifted myelopoiesis, granulocytic hyperplasia, increased ratio of myeloid cells to erythroid cells, increased megakaryocytes
- Myeloblasts comprise less than 5% of marrow cells
- The HALLMARK of the disease is the bcr/abl gene (philadelphia chromosome) that is detected by the PCR test in the peripheral blood and bone marrow.
35
Lab to diagnose blast phase of CML
when blasts are >20% of bone marrow
36
What happens to spleen in sickle cell patients
There will be splenic ATROPHY (autosplenectomy)
splenic sequestration happens in infancy/childhood therefore you should NOT be able to palpate a spleen in adult patients with SSD.
37
DVT workup
D dimer
Compression US
Contrast venography is gold standard but painful, invasive
MRDTI
38
What causes acute hemolytic blood transfusion reaction
involves incompatible mismatches in ABO system that are isoagglutinin-mediated; severity depends on dose of RBCs given, most severe seen in surgical pts under anesthesia
39
Signs and symptoms of patient with hemolytic blood transfusion reaction
- awake: fever, chills, backache, headache, apprehension, dyspnea, hypotension, cardiovascular collapse, pain at infusion site, chest pain, dizziness, bronchospasm
- Under general anesthesia: tachycardia, generalized bleeding, oliguria; Severe cases: acute DIC, acute kidney injury (AKI) from acute tubular necrosis (ATN), death in 4% due to ABO incompatibility
40
Labs in acute hemolytic blood transfusion reaction
- Decreased Hct and serum haptoglobin; hgb will fail to rise by expected amount
- evidence of AKI or acute DIC (monitor coags)
- recipient plasma-free Hgb elevated resulting in hemoglobinuria (wine-colored urine) and plasma hemoglobinemia (pink plasma)
- elevated LDH, indirect bilirubin, creatinine
41
Treatment acute hemolytic blood transfusion reaction
stop transfusion, vigorously hydrate (NS or suitable crystalloid) to prevent ATN and maintain UOP >100mL/hr until hypotension corrected and hemoglobinuria clears, forced diuresis with mannitol (controversial) may prevent or minimize AKI, monitor VS, maintain airway
42
Cause of delayed hemolytic blood transfusion reaction
minor RBC antigen discrepancies; less severe; mediated by IgG antibodies causing extravascular RBC destruction; may occur 5-10 days post-transfusion; most common antigens are Duffy, Kidd, Kell, C/E loci of Rh system
43
Labs for delayed hemolytic blood transfusion reaction
Unexpected drop in Hgb and increased total & indirect bilirubins; new offending alloantibody detected in pt’s serum; Direct antiglobulin test positive, indirect COOMBS TEST POSITIVE
44
Treatment for delayed hemolytic blood transfusion reaction
stop transfusion, vigorously hydrate (NS or suitable crystalloid) to prevent ATN and maintain UOP >100mL/hr until hypotension corrected and hemoglobinuria clears, forced diuresis with mannitol (controversial) may prevent or minimize AKI, monitor VS, maintain airway
45
What is Transfusion-related acute lung injury (TRALI)
Noncardiogenic pulmonary edema after blood transfusion without other explanation; most susceptible in surgical and critically-ill patients
46
What causes TRALI
associated with allogeneic antibodies in donor plasma components that bind to recipient leukocyte antigens
47
Treatment and prevention of TRALI
No treatment, only supportive.
| To prevent, use male-only plasma donors
48
Cause of blood transfusion bacterial contamination
Due to blood products, especially platelets, prone to bacterial contamination because they cannot be refrigerated
49
Signs and symptoms of blood transfusion bacterial contamination-gram positive
fever, bacteremia, rarely causes sepsis syndrome
50
Signs and symptoms of blood transfusion bacterial contamination-gram negative
Fatal ! septic shock, acute DIC, AKI due to endotoxin
51
Prevention of blood transfusion bacterial contamination-gram negative
enhanced venipuncture site skin cleansing, diverting first few mLs of donated blood, use of single-donor blood products (as opposed to pooled-donor), point-of-care rapid bacterial screening to discard questionable units
52
Treatment for blood transfusion bacterial contamination
Culture both the pt and donor blood bag; give antibiotics immediately
53
Hallmark of ALL
pancytopenia with circulating blasts
54
CBC of ALL
normochromic, normocytic anemia, thrombocytopenia
55
What is on peripheral smear for ALL
lymphoblasts
56
Initial workup of ALL
CBC, peripheral smear, assess basic organ function (creatinine, bilirubin), blood glucose (glucocorticoids are part of therapy), spontaneous tumor lysis syndrome (K+, CA++, PO₄++, uric acid)
57
When to do Coag studies for ALL
Coag studies (full DIC screen) prior to lumbar puncture
58
Treatment for INR: No significant bleed, INR above therapeutic range but <5
lower or omit next dose, monitor more frequently and resume at lower dose when INR falls within therapeutic range
59
Treatment for INR: No significant bleed, INR >5 but <9
- Hold next 1-2 doses, monitor more frequently and resume at lower dose when INR falls within therapeutic range
- Patients at high risk for bleeding→ hold and consider giving vitamin K 1-2.5 mg orally, recheck INR in 24-48 hours
60
Treatment for INR: No significant bleed, INR >9
Hold coumadin, Vitamin K 2.5-5 mg orally, monitor frequently and resume at lower dose when INR falls within therapeutic range
61
Treatment for INR: Patient has serious/life threatening bleed
Hold coumadin and give 10 mg Vitamin K by slow IV infusion supplemented by FFP, PCC, or recombinant factor VIIa (PCC preferred)
62
When to monitor LMWH patients
If patient has moderate kidney disease, elevated BMI, low weight, select pregnant patients monitor using anti-Xa activity level
63
Why is LMWH preferred for DVT
preferred due to ease of administration, less hemorrhage, and significantly fewer deaths. Lower incidence of HIT. Better control for CA patients
64
Use caution when giving LMWH to whom?
LMWH is predominantly excreted in the urine. Must be used with caution in those with creatinine clearance <30 ml/min
65
Unfractionated heparin mechanism of action
binds to antithrombin and enhances its ability to inhibit the body's clotting factors. Prevents fibrin formation and inhibits thrombin-induced activation of platelets and factors
66
LMWH mechanism of action
produced from chemical depolymerization of unfractionated heparin. reduced inhibitory activity against thrombin
67
Fondaparinux mechanism of action
exerts no thrombin inhibition. Indirectly inhibits factor Xa through binding to antithrombin
68
Vitamin K antagonist (Coumadin) mechanism of action
inhibits the activity of the vitamin K-dependent carboxylase that is important for the modification of factors II, VII, IX, X
69
direct factor Xa inhibitor mechanism of action (dabigatran, rivaroxaban, apixaban, edoxaban)
inhibits clotbound and free thrombin and thrombin induced platelet aggregation
70
Labs for thrombocytosis
elevated platelet count (>450,000/ml, can be over 2,000,000/mcl) in peripheral blood, Normal RBC mass, WBC mildly elevated (<30,000), normal hematocrit, Large platelets
71
Bone marrow for thrombocytosis
increased megakaryocytes but no morphologic abnormalities
72
How to differentiate thrombocytosis from CML
No bcr/abl present, BUT Philadelphia chromosome should be assayed to r/o out CML differential
73
What is polycythemia vera
disorder of the myeloid/erythroid stem cell resulting in erythropoietin-independent proliferation of erythrocytes.
74
Labs for polycythemia vera
- LOW serum erythropoietin
- Elevated RBC count (>6 million)
- Elevated Hgb (>18 in men, >16 women)
- Elevated Hct (<54% in men, <49% in women)
- Increased WBC (with basophilia)
- Thrombocytosis (can be > 100,000,000/mcl)
- May have JAK2 mutation
75
Major Criteria for Diagnosis of Polycythemia vera
hemoglobin level > 18.5 g/dl for men or > 16.5 g/dl for women
Presence of JAK2 V617F or similar mutation
76
Minor Criteria for Diagnosis of Polycythemia vera
- Bone Marrow trilineage myeloproliferation
- Subnormal serum erythropoietin level
- Endogenous erythroid colony formation in vitro
77
When is there low o2 st with polycythemia vera?
- Secondary if splenomegaly is absent, high hematocrit not accompanied by increases in other cell lines. Can be caused by hypoxemia, erythropoietin producing states, stress polycythemia (gaisbock’s syndrome), hemoglobinopathies
- All have arterial hypoxemia (low o2sat !)
78
Hemochromatosis risk factors
European descent, inheritance of two mutated HFE genes, alcoholism
79
Hemochromatosis labs
Transferrin >45%, elevated serum ferritin, HFE gene mutation, hyperglycemia, abnormal AST and alk phos)
80
Hemochromatosis SandS
- Early: nonspecific (fatigue, arthralgia)
- Late: arthropathy, hepatomegaly, liver dysfunction, skin pigmentation (combo of slate blue and brown, making bronze), cardiac enlargement, diabetes, erectile dysfunction
81
Hemochromatosis treatment (non-medicine)
Avoid foods rich in iron, alcohol, vitamin c, raw shellfish, and supplemental iron
Weekly phlebotomy of 1-2 units of blood for 2-3 years (to deplete iron stores), then every 2-4 months
82
Hemochromatosis treatment (medications)
- Deferoxamine (Desferal) IV 20-40 mg/kg/d infused over 9-12 hrs (painful and time consuming)
- Derasirox PO 20 mg/kg, once daily; used for iron overload due to blood transfusions; Can cause renal impairment, hepatic impairment, or GI hemorrhage
- Deferiprone PO 25 mg/kg, TID
83
Hemochromatosis complications
- Liver Cirrhosis: Hepatoma – only in pts with liver cirrhosis
- Diabetes Mellitus
- Cardiomyopathy
- Arthritis
- Hypogonadism
84
CML chromosome
Presence of brc/abl gene (philadelphia chromosome)
85
CML best initial therapy
Tyrosine kinase inhibitor (TKI): imatinib, dasatinib, nilotinib
86
Hemophilia SandS
- Spontaneous or trauma induced bleeding
- Bleeding most commonly seen in joints, hot, swollen, painful joints; crippling painful deformity
- Bleeding in GI tract and muscles
- Compartment syndrome
- Hematuria
87
Hemophilia labs
- PTT prolonged
- PT normal
- PTT mixing study will fully correct
- Reduced factor VIII
- Normal factor VIII antigen, fibrinogen level, and bleeding time
88
Ann Arbor stage 1
cancer is located in a single region, usually one lymph node and the surrounding area. Stage I often will not have outward symptoms
89
Ann Arbor stage 2
the cancer is located in two separate regions, an affected lymph node or lymphatic organ and a second affected area, and that both affected areas are confined to one side of the diaphragm—that is, both are above the diaphragm, or both are below the diaphragm.
90
Ann Arbor stage 3
the cancer has spread to both sides of the diaphragm, including one organ or area near the lymph nodes or the spleen.
91
Ann Arbor stage 4
diffuse or disseminated involvement of one or more extra-lymphatic organs, including any involvement of the liver, bone marrow, or nodular involvement of the lungs
92
Infections that are common in those with impaired cellular immunity
- Bacteria: listeria, legionella, salmonella
- Myobacterium: herpes simplex, varicella, CMV
- Fungi: cryptococcus, coccidioides, histoplama, pneumocystis
- Protozoa: toxoplasma
93
How do treat metastatic bone pain
external beam RT (EBRT), radioisotopes and targeted therapy given in association with analgesics (NSAIDS and opioids) have an important role in bone pain management
94
Meds that cause thrombocytopenia
- Chemotherapy
- Antiplatelets: ex abciximab, anagrelide, eptifibatide
- Antimicrobials: ex adefovir, flucanozole, isoniazid, linezolid, PCN, rifampin, vanco, sulfa drugs
- Cardiovascular agents: ex amiodarone, atorvastatin, digoxin, hydrochlorothiazide
- GI agents: ex cimetidine, famotidine, ranitidine
- Neuropsychiatric agents: ex carbamazepine, Haldol, methyldopa, phenytoin
- Analgesic agents: ex Tylenol, ibuprofen, diclofenac, naproxen
- Anticoagulant agents: ex heparin, LMWH
- Immunomodulator agents: ex interferon alpha, rituximab
- Immunosuppressant agents: mycophenolate mofetil, tacrolimus
- Other agents: immunization, contrast dye
95
How much does 1 unit of platelets raise platelet count
1 unit of platelets raises platelet count by 50,000 to 60,000 platelets/mcL
96
How long do transfused platelets last
Transfused platelets last 2-3 days
97
Indication for platelet transfusion
- Thrombocytopenia due to decreased platelet production.
- Give prophylactically when there is active bleeding and platelet count <10,000/mcL
- Also given when there will be invasive procedure or surgery in thrombocytopenic patients
98
How is cross matching performed
- Involves mixing the donor's RBCs and serum with the serum and RBCs of the recipient to identify the potential for a transfusion reaction. The end point of all crossmatches is the presence of RBC agglutination (either gross or microscopic) or hemolysis.
- Full cross matching takes about 45 minutes.
99
Common acid base disturbance with blood transfusion
HYPOcalcemia
100
INR of FFP
about 1.6; INR won't drop below 1.3 if getting FFP, may need vitamin K too if treating warfarin overdose
101
What factors are in FFP
about 1 unit/mL of all coagulation factors
102
What factors are in cryoprecipitate
fibrinogen, factor VIII, and von Willebrand factor
103
What factors are in Prothrombin complex concentrate
Factor II, Factor VII , Factor IX, Factor X
104
When will you admit a pt with hemophilia A to the hospital?
- Bleeding unresponsive to outpatient tx
| - Major invasive procedures b/c of the need for serial infusions of clotting factor concentrate
105
Acute cellular rejection diagnosis and treatment
- Symptoms dependent on organ, biggest symptom is graft tenderness
- Typically occurs within first few months, but can occur at any time
- Treatment: High dose IV corticosteroids. If no improvement then give cytolytic therapy
106
When is therapeutic phlebotomy indicated?
When there is there is too much (Hemochromatosis) iron or red blood cells in the body
