Hematology Flashcards
(137 cards)
1
Q
Best initial test when concern for anemia
A
CBC with peripheral smear
2
Q
What are supplementary tests to order on CCS when concerned about anemia?
A
Reticulocyte count, Iron labs, TSH and T4, haptoglobin, bilirubin, LDH, B12/folate
3
Q
What type of anemia is caused by blood loss?
A
Iron deficiency
4
Q
What are iron labs in iron deficiency anemia?
A
Iron: Low FERRITIN: LOW TIBC: High Transferrin saturation: Low Elevated RDW
5
Q
Most accurate test to dx IDA
A
Bone marrow biopsy (do not order on CCS)
6
Q
Tx for IDA
A
Oral ferrous sulfate
7
Q
Iron studies in anemia of chronic disease
A
Iron: Low
FERRITIN: HIGH
TIBC: Low
Transferrin saturation: Low or normal
8
Q
Buzzword
Microcytic anemia with target cells
A
Thalassemia
9
Q
Most accurate test for dx of thalassemia
A
Hemoglobin electrophoresis
(Beta form: elevated HgA2 and HgF)
(Alpha form: normal)
10
Q
Is sideroblastic anemia microcytic or normocytic?
What are potential causes (3)?
A
Microcytic
Alcohol, Isoniazid, Lead exposure
11
Q
Iron levels in sideroblastic anemia are ….
A
High
12
Q
Most accurate test for dx of sideroblastic anemia
A
Prussian blue stain
13
Q
Alpha thalassemia is most accurately diagnosed by …
A
DNA sequencing
14
Q
What is one of the only anemias with a high RDW and why?
A
IDA
As there is less iron the RBCs made are smaller and smaller which leads to large variation in size
15
Q
Besides fatigue what else does B12 deficiency anemia present with?
A
Peripheral neuropathy but can be other neurologic problems like dementia
16
Q
Buzzwords
Glossitis and diarrhea associated anemia
A
B12 deficiency
17
Q
Buzzwords
Hypersegmented neutrophils and oval cells
A
Macrocytic anemia
18
Q
What happens to reticulocyte count in B12 deficiency anemia?
A
Low
B12 deficiency causes destruction of cells just as they leave bone marrow which causes low reticulocyte count
19
Q
What happens to homocysteine and methylmalonic acid levels in B12 and folate deficiency?
A
B12: homocysteine and methylmalonic acid levels rise
Folate: homocysteine levels rise
20
Q
You’ve confirmed a low B12 and high methylmalonic acid. What is the next test you should run for etiology?
A
Antiparietal cell Abs and anti-intrinisic factor Abs to look for pernicious anemia
21
Q
Hemolytic anemia labs
Indirect bili, reticulocyte count, LDH, haptoglobin, PBS
A
Indirect bilirubin: High Reticulocyte count: High LDH: High Haptoglobin: Low Spherocytes on PBS
22
Q
What should you order for the PE on CCS for sickle cell anemia?
A
Complete physical exam
23
Q
What are best initial therapies to offer a patient presenting with sickle cell anemia pain?
A
Oxygen, pain meds, fluid hydration
24
Q
Is fever in sickle cell anemia a emergency?
What medication should you give?
A
Yes
Levofloxacin, moxifloxacin, or ceftriaxone
25
What should be repleted in all sickle cell patients?
Folate
26
Most accurate test for parvovirus B19 dx in sickle cell patients
Parvovirus DNA PCR
27
Parvovirus infection in sickle cell is best treated how?
Transfusions and IVIG treatment
28
At discharge what should sickle cell patients be discharged on?
Folate
Hydroxyurea
Pneumococcal vaccination
29
What is the only manifestation of hemoglobin SC disease?
Renal: hematuria, UTI, isosthenuria (inability to concentrate urine)
30
Most accurate diagnostic test for autoimmune hemolysis
Coomb's test
31
Best initial therapy for autoimmune hemolysis
Steroids (prednisone)
32
If there are recurrent episodes of hemolysis in autoimmune hemolysis then what is the most effective therapy?
Splenectomy
33
Are 'warm Abs' IgM or IgG?
IgG
*Only IgG abs respond to steroids and splenectomy
34
Buzzword
Mycoplasma or EBV associated anemia
Cold-induced hemolysis
35
What will be the result of a standard Coomb's test in cold-induced hemolysis? What test will be positive?
Negative
Complement test will be positive
36
How is cold-induced hemolysis treated?
Rituximab
*No response to steroids, splenectomy, or IVIG
37
Is cold-induced hemolysis IgG or IgM?
IgM
38
What is mode of inheritance of G6PD deficiency?
X-linked recessive
39
What are common triggers of G6PD deficiency anemia?
Infection
Oxidizing drugs (e.g. sulfas, primaquine, dapsone)
Fava beans
40
Buzzwords
Heinz bodies and bite cells are seen in this anemia
G6PD deficiency
41
What is the most accurate test for dx of G6PD deficiency and when should it be ran?
G6PD level but should be done 2 months after the episode. If done too early it will be normal bc the most deficient cells will have been destroyed
42
Recurrent episodes of hemolysis, splenomegaly, bilirubin gallstones, and a high MCHC are signs of which anemia cause?
Hereditary spherocytosis
43
What is the most accurate diagnostic test for hereditary spherocytosis?
Osmotic fragility test
44
Tx of hereditary spherocytosis
Splenectomy
45
Buzzword
E. Coli 0157:H7
Hemolytic-Uremic Syndrome
46
Ticlodipine can predispose to what macroangiopathic hemolytic anemia?
Thrombotic thrombocytopenic purpura (TTP)
47
Findings in HUS
Intravascular hemolysis
Elevated BUN and creatinine
Thrombocytopenia
48
Findings in TTP (5)
```
Intravasacular hemolysis
Elevated BUN and creatinine
Thrombocytopenia
Fever
Neurological abnormalities
```
49
ADAMTS-13 level in TTP is ...
Low
50
Should you transfuse platelets in a patient with TTP or HUS?
No
51
Severe cases of TTP or HUS are treated with what?
Plasmapharesis
52
Patient presents with pancytopenia and dark urine only in am. Dx?
Paroxysmal nocturnal hemoglobinuria
53
What is most common cause of death in paroxysmal nocturnal hemoglobinuria?
Large vessel thrombosis
54
Most accurate test for diagnosis of PNH
CD55 or CD59 Ab (aka decay accelerating factor)
55
Best initial tx of PNH
Steroids
*Transfusion-dependent patients with severe disease are treated with eculizumab
56
What are some drugs which may cause methemoglobinemia?
Nitroglycerin, amyl nitrate, nitroprusside dapsone, -caines
57
What may cause SOB with clear lungs on exam, normal CXR, and brown blood? What is tx?
Methemoglobinemia
| Methylene blue
58
A patient receiving blood transfusion and becoming anaphylactic with normal LDH and bilirubin is caused by ...
IgA deficiency
59
Delayed jaundice in a patient receiving a blood transfusion is caused by ...
Minor blood grouping incompatability
60
Buzzwords
Auer rods associated with
AML
61
Buzzwords
DIC associated leukemia
M3, acute promyelocytic leukemia
62
Best initial test in diagnosis of acute leukemia
PBS showing blasts
63
What is the most important prognostic indicator in acute leukemia which may be an indication for bone marrow transplantation in addition to chemo?
Cytogenetic abnormalities suggesting high rate of relapse
64
Chemo agents used in acute leukemia are?
What is added in M3 (acute promyelocytic) leukemia?
What is added for ALL?
Idarubicin (or daunorubicin) and cytosine arabinoside
In M3 add all-trans retinoic acid
In ALL add intrathecal MTX
65
In patients with extremely high WBC count in leukemia what is the recommended therapy?
Leukapheresis and hydroxyurea to reduce WBC count
66
What presents with pancytopenia, elevated MCV, low reticulocytes, and macroovalocytes?
Myelodysplasia
67
Are there more or less blasts in myelodysplasia compared to leukemia?
Less. Myelodysplasia is like a preleukemia syndrome which is slower and may ultimately progress to leukemia
68
Buzzword
Pelger-Huet cells association
Myelodysplasia
*The cell is a neutrophil with two lobes
69
What is the most common cause of death in patients with myelodysplasia?
Infection or bleeding
70
Tx of myelodysplasia
Supportive with transfusions as needed
*Azacitadine (increases survival), decitabine, and lenalidomide
71
Why do patients have early satiety in CML and CLL?
Splenomegaly may compress stomach
72
What happens to the following values in CML?
Leukocyte alkaline phosphatase (LAP)
Neutrophils
LAP decreases
| Neutrophils increase
73
Elevated LAP score indicates ...
Leukomoid reaction (reactive increase in WBC)
74
Buzzword
Philadelphia chromosome
bcr/abl in CML responding to Gleevac (imatinib)
75
Best initial therapy CML
Imatinib
*BM transplantation cures CML but is never the best initial test
76
Best initial test for CLL
PBS showing lymphocyte smudge cells
77
Tx for CLL
Stage based. Early forms of disease do not need treatment.
Advanced stage disease: fludarabine (prolongs survival), rituximab, and cyclophosphamide
78
Should you treat elevated WBC count (asymptomatic) in patients with CLL?
No
79
If fludarabine fails in CLLthen what med is used?
Alemtuzumab (anti-CD52 agent)
80
Smear showing hairy cells and immunophenotyping are the most accurate tests/findings for what disease?
Hairy cell leukemia
81
Best initial therapy in hairy cell leukemia
Cladribine (2-CDA)
82
How can myelofibrosis and hairy cell leukemia be distinguished?
Hairy cell leukemia has elevated TRAP
| Normal TRAP in myelofibrosis
83
Buzzword
Tear drop cells
Myelofibrosis
84
What mutation is seen in myelofibrosis?
JAK2
*Ruxolitinib inhibits JAK2 and is occasionally used
85
Best initial therapy for myelofibrosis
Lenalidomide or thalidomide
86
Headache, blurred vision, dizziness, and pruritis may be seen in what disorder?
Polycythemia vera
87
MCV in polycythemia vera
Low
88
JAK2, normal O2 sat, and low MCV are seen in ...
Polycythemia vera
89
Best initial therapy for polycythemia vera
Phlebotomy
90
Meds used for polycythemia vera and essential thrombocythemia
Hydroxyurea (lowers cell count)
Aspirin
Anagrelide (if thrombocythemia)
91
What is erythromelalgia?
Pain in hands and feet seen in erythrocythemia from high platelet count
92
What are most common causes of death of multiple myeloma?
Renal failure and infection
93
Elevated levels of monoclonal IgG antibody on SPEP may suggest what disease?
Multiple myeloma
94
Buzzwords
Bence-Jones protein on UPEP
Multiple myeloma
95
How will a peeripheral smear appear in multiple myeloma?
Rouleaux formation from RBC clumping in chains
96
What is a prognostic indicator in multiple myeloma?
Beta-2 microglobulin
97
What medicine reverses the renal dysfunction in multiple myeloma?
Bortezomib
98
B12 and LAP levels are changed how in polycythemia vera?
Both elevated
99
What is the most specific test for diagnosis of multiple myeloma?
Bone marrow biopsy
100
Tx of multiple myeloma
Melphalan and steroids
| Thalidomide, bortezomib, and lenalidomide may be added
101
Patients that are relatively young (
Autologous stem cell BM transplantation
102
Patients with hypercalcemia in multiple myeloma should be managed how?
Hydration and diuresis
103
Asymptomatic elevation of IgG on SPEP
Monoclonal gammopathy of unknown significance
104
What are the best initial tests in Waldenstrom's Macroglobulinemia?
Serum viscosity test
| SPEP with elevated IgM
105
Best initial therapy for symptomatic patients with Waldenstrom's
Plasmapharesis
| additional tx are rituximab fludarabine, chlorambucil, or rituximab
106
Chronic Hep B and C can cause ...
Aplastic anemia
107
What are therapy options for patients with aplastic anemia?
If young and a match BM transplant. If not either then antithymocyte globulin and cyclsporine
108
How are Hodgkin's disease and Non-Hodgkins's disease different in terms of disease localization?
Hodgkin's: Starts centrally, near cervical region and spreads out from center
Non-Hodgkin's: Widespread
109
What is the best initial test for diagnosing Hodgkin's and NHL?
Excisional lymph node biopsy
110
Histologically how do Hodgkin's and NHL differ
Hodgkin's has Reed-Sternberg cells (look like owls)
111
Describe the staging scheme for lymphoma
Stage I: Single lymph node group
Stage II: Two lymph node groups on one side of diaphragm
Stage III: Lymph node groups on both sides of diaphragm
Stage IV: Widespread disease
112
What other tests are helpful in staging lymphoma?
CXR
BM biopsy
CT scan with contrast
113
How are the different stages of lymphoma treated in general terms (e.g. radiation, chemo, etc)?
Stages I and II: Radiotherapy and chemo
| Stage III and IV: Chemo
114
Tx for Hodgkin's Disease
ADVB
| Adriamycin, bleomycin, vinblastine, dacarbazine
115
Tx for NHL
```
CHOP
Cyclophosphamide
Hydroxyadriamycin
Oncovin (vincristine)
Prednisone
```
If anti-CD20 positive then add Rituximab
116
Von Willenbrand's Disease is caused by what abnormality? What happens to platelet and clotting times?
Platelet dysfunction from lack of vWF
Platelet counts are normal
PTT may be elevated because vWF stabilizes factor VIII
117
What are the most accurate tests for diagnosing vWD?
Ristcetin cofactor assay (can tell if vWF is working even if normal)
vWF level
118
What is first-line treatment for vWD?
Desmopression (DDAVP)
119
If DDAVP does not work for vWD then what is another treatment option?
Factor VIII replacement (contains both vWD and factor VIII)
120
Antibodies to what receptor cause ITP?
Antibodies to glycoprotein IIb/IIIa receptor
121
Peripheral smear demonstrating large platelets and BM showing megakaryocytes may be indicative of what disorder?
ITP
122
If ITP is suspected what is the best next step?
Provide prednisone. It is a diagnosis of exclusion and time should not be spent trying to diagnosis
123
In severe ITP presenting low platelet count (e.g.
Provide IVIG
124
What do romiplostim and eltrombopag do?
They're thrombopoietin analogs which directly stimulate megakaryocytes and can be used to treat chronic ITP
125
Normal ristocetin and vWF levels in a patient with platelet-type bleeding and kidney failure indicates what problem?
Uremia-induced platelet dysfunction
126
What is specifically disrupted in platelets from patients with uremia-induced platelet dysfunction?
Their ability to degranulate
127
Tx of uremia-induced platelet dysfunction
DDAVP, dialysis, estrogen
128
What is treatment for factor XII deficiency?
No treamtent needed
129
What is treatment for factor XI deficiency?
Fresh frozen plasma
130
Does thrombosis in HIT occur more in veins or arteries?
Veins
131
Best initial diagnostic tests for HIT
Platelet factor 4 Abs or heparin-induced platelet antibodies
132
Buzzword
Russel viper venom test
Most accurate test for lupus anticoagulant
133
What are clotting times in antiphospholipid syndrome and other unique lab results?
Elevated aPTT
Normal PT
False positive VDRL
134
Tx of APAS
Heparin followed by warfarin
135
How does protein C deficiency present when starting anticoagulation?
Skin necrosis when patients go on warfarin
| Venous thrombosis
136
What is treatment of protein c deficiency?
Heparin followed by warfarin
137
What is MOA of heparin?
Inactivates thrombin and activated Xa
