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Step 3 COPY > MTB - Hematology > Flashcards

MTB - Hematology Flashcards

1
Q

In older patient with anemia - what level of Hb is your goal?

A

Above 10

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2
Q

Iron studies in anemia of chronic disease

A

High ferritin
Low TIBC
Low Fe
Normal/low Fe sat

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3
Q

Electrophoresis in B thalassemia

A

Elevated HbA2 and HbF

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4
Q

Hb electrophoresis in A-thalassemia

A

Normal

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5
Q

What are potential causes of sideroblastic anemia?

A

Alcoholic
Lead exposure
Isoniazid

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6
Q

Most accurate diagnostic test for sideroblastic anemia

A

Prussian blue stain

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7
Q

Treatment of sideroblastic anemia

A
  1. Remove toxin exposure

2. Pyridoxine replacement

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8
Q

Most accurate test for alpha thalassemia

A

DNA sequencing

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9
Q

Which anemia has increased RDW?

A

Iron deficiency only

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10
Q

Only microcytic anemia with high reticulocyte count

A

HbH - 3 alpha gene deletion

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11
Q

Anemia of chronic disease

A

Normal or increased amounts of iron and ferritin in storage but inability to process the iron into a usable form for Hb synthesis

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12
Q

EPO can be used to tx what anemia?

A

Anemia of chronic disease due to end stage renal disease

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13
Q

Three unique signs of vit B12 deficiency

A

Neuropathy (peripheral)
Glossitis (smooth tongue)
Diarrhea

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14
Q

Which drug can block vit B12 absorption?

A

Meteor in

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15
Q

Peripheral blood smear findings in megaloblastic anemia

A 
Hypersegmented neutrophils (>4 lobes) 
Oval cells
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16
Q

What other lab tests should you get in macrocytic anemia?

A

Bilirubin and LDH - will be increased

Reticulocyte count - will be decreased

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17
Q

Your suspect vit B12 deficiency in your patient with macrocytic anemia, but their b12 level is normal - what other test can help you distinguish between vitamin B12 and folate deficiency?

A

Methylmalonic acid level - will be elevated in B12 def

Homocysteine is elevated in both

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18
Q

You found low vitamin B12 levels and elevated methylmalonic acid levels in your patient - what’s the next test to determine etiology of deficiency?

A

Anti-parietal cell abs or anti- intrinsic factor abs

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19
Q

Lab findings in hemolytic anemia

A

Elevated bilirubin, LDH and reticulocytes

Decreased haptoglobin

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20
Q

In addition to regular findings of hemolysis, intravascular hemolysis shows…

A

Schistocytes, helmet cells and fragmented cells on smear
Hemoglobinuria
Hemosiderin urea

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21
Q

A patient with sickle cell anemia presents with severe pain in the chest, back and thighs. What is your initial management?

A

Oxygen
IVF with normal saline
Pain meds

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22
Q

Patient with sickle cell crises, develops fever while on oxygen and hydration. What do you do?

A

Give abx: ceftriaxone, levofloxacin or moxifloxacin
Perform complete physical exam
Order blood cultures, urinalysis, reticulocyte count, CBC and CXR

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23
Q

In what cases would you give an exchange transfusion to a sickle cell pt

A

Visual disturbance
Pulmonary infarction
Priapism
Stroke

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24
Q

Sickle cell pt admitted for pain crisis has a drop in htc with rise in reticulocyte count over two days - what do you suspect?

A

Either folate deficiency or parvovirus infection

If pt is receiving replacement therapy, it is likely parvo

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25
Q

Dx and Tx of parvovirus in sickle cell pt

A

PCR for DNA of parvovirus

Tx, transfusion, IVIG

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26
Q

What meds do you discharge a sickle patient?

A

Folate replacement
Pneumococcal vaccine
Hydroxyurea - if >4 episodes per year

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27
Q

CF in sickle cell trait

A

Renal problems

  • hematuria
  • isostenuria (prone to dehydration)
  • urinary tract infections
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28
Q

Causes of autoimmune hemolysis

A

Other autoimmune conditions:SLE, RA
CLL
MEDS: penicillin, alpha-methyl dopa, quinine, sulfa drugs

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29
Q

Most accurate diagnostic test in autoimmune hemolysis

A

Coombs test

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30
Q

Best initial therapy in autoimmune hemolysis

A

Steroids (prednisone)
If recurrent - splenectomy
Only IgG abs respond to these treatments

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31
Q

If a case describes severe hemolysis that does not respond to prednisone or repeated transfusions - what can you do?

A

Give IVIG

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32
Q

Features of cold induced hemolysis

A

Mycoplasma or EBV in history
Coombs negative
Complement positive
No response to steroids, transfusion or IVIG

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33
Q

Tx. Cold hemolysis

A

Rituximab

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34
Q

Which anemia is X-linked?

A

G6PD Deficiency

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35
Q

Causes of acute oxidant stress in g6pd deficiency

A

Infection
Sulfa drug, primaquine, dapsone
Fava beans

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36
Q

Best initial test in g6pd deficiency

A

Heinz body test - Heinz bodies and bite cells on smear

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37
Q

Most accurate test for G6PD deficiency

A

G6pd level - wait two weeks after acute episode to measure level (will be normal right after episode )

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38
Q

Tx. G6PD deficiency

A

No tx - avoid oxidant stress

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39
Q

CF in hereditary spherocytosis

A

Recurrent episodes of hemolysis
Splenomegaly
Bilirubin gallstones
Elevated MCHC

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40
Q

Causes of HUS / TTP

A

E.coli O157:H7

Ticlopidine

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41
Q

HUS triad

A

Hemolysis
Renal failure
Thrombocytopenia

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42
Q

TTP

A 
Hemolysis
Renal failure 
Thrombocytopenia 
Fever
Neurological changes
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43
Q

Tx. HUS / TTP

A

Plasmapheresis

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44
Q

Patient is presenting with pancytopenia and recurrent episodes if dark urine - dx? Test?

A

Dx: PNH

Test - c55 and c59 ab level

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45
Q

Tx. PNH

A

Steroids

If transfusion dep. try eculizumab

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46
Q

What drugs can cause methemoglobinemia?

A 
Nitroglycerin 
Amyl nitrate
Nitroprusside
Dapsone
Anesthetics ending in a sine
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47
Q

Tx. Methemoglobinemia

A

Methylene blue

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48
Q

Acute symptoms of hemolysis while transfusion is occuring

A

ABO incompatibility

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49
Q

Acute SOB shortly after transfusion that goes away on its own

A

Transfusion assoc. Acute lung injury

  • donor abs against WBCs
  • no tx
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50
Q

What transfusion reaction presents with delayed jaundice and no other symptoms?

A

Minor blood group incompatibility

No specific therapy

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51
Q

Best initial test if you suspect leukemia

A

Peripheral smear - will show blasts

52
Q

Most impt prognostic test in leukemia

A

Cytogenetics ex. Karyotypic analysis

53
Q

Tx. AML

A

Chemotherapy with idarubicin and cytosine arabinoside

54
Q

What extra tx. Is given in M3 AML

A

All trans retinoic acid

55
Q

Additional therapy for ALL

A

Intrathecal methotrexate

56
Q

Leukostasis as the initial presentation of acute leukemia

A

Slushing of blood in blood vessels of eyes, brain and lungs when WBC is > 100,000.

57
Q

Tx. Leukostasis

A

Leukaphoresis

Hydroxyurea

58
Q

Pelger-Huet cell

A

Neutrophil with two lobes seen in myelodysplasia

59
Q

Pt presents with pancytopenia, elevated MCV, low retic count and macroovulocytes with Pelger Huet cells. Dx?

A

Myelodysplasia

60
Q

Tx. Myelodysplasia

A

Supportive, transfusions as needed

Azacitadine - specific therapy

61
Q

Lenalidomide

A

Drug approved for 5q minus syndrome form of myelodysplasia

62
Q

Pt presents with elevated WBC predominantly neutrophils and splenomegaly - dx?

A

CML

63
Q

Best initial test in CML

A

LAP score

- elevated neutrophils but LOW LAP

64
Q

Most accurate test for cml

A

Philadelphia xm

65
Q

Tx. CML

A

Imatinib (also can use: dasatinib or nilotinib)

BMT only if no response to medical therapy

66
Q

55 year old patient presents with elevated WBC on routine CBC. It is predominantly lymphocytes. Dx? Best initial test?

A

Dx. CLL

TEST: peripheral smear will show smudge cells

67
Q

Tx. CLL

A

Based on stage
Stage 0-1: elevated cells, enlarged LN - no therapy
Stage 2-4: splenomegaly, anemia, low platelets - fludarabine + rituximab

68
Q

Alemtuzumab

A

Anti CD52 AB used in tx of CLL

69
Q

55 yo pt presents with pancytopenia and massive splenomegaly. dx? Test?

A

Dx. Hairy cell leukemia

Test: trap stain, peripheral smear shows hairy cells

70
Q

Tx. Hairy cell leukemia

A

Cladribine (2-CDA)

71
Q

55 yo patient presents with pancytopenia and splenomegaly. TRAP stain is negative. Smear shows tear drop cells. Dx? Tx?

A

Myelofibrosis
Tx. BMT
If BMT is not possible, best initial therapy is Lenalidomide or thalidomide

72
Q

Lab findings in polycythemia Vera

A

Elevated Htc, WBC and platelets

Decreased MCV and EPO

73
Q

A patient has high hematocrit on CBC, what test do you order next?

A

Arterial blood gas
- want to rule out hypoxia as a cause of erythrocytes is
EPO level
Jak2 mutation

74
Q

Tx. Polycythemia Vera

A

Phlebotomy
Hydroxyurea - can decrease red count
Daily aspirin

75
Q

Tx. Multiple myeloma

A

Melphalan and steroids

76
Q

Most effective therapy for multiple myeloma

A

Autologous BMT

77
Q

Prognostic indicator in multiple myeloma

A

Beta 2 micro globulin level

78
Q

MGUS

A

Asymptomatic elevation of IgG in elderly person with total elevated protein. No treatment

79
Q

Pt presents with blurry vision, confusion, headache, enlarged LN and spleen. IgM levels are elevated - dx

A

Waldenstrom macroblobulinemia

80
Q

Best initial test in Waldenstrom macroglobulinemia

A

Serum viscosity test or SPEP

81
Q

Best initial therapy for Waldenstrom macroglobulinemia

A

Plasmapheresis

82
Q

Best initial test for Hodgkins and NHL

A

Excisional lymph node biopsy

83
Q

Chemo tx for hodgkins

A

Adriamycin
Bleomycin
Vinblastine
Dacarbazine

84
Q

Chemotherapy for NHL

A 
Chop regimen
Cyclophosphamide 
Hydroxyadriamycin
Oncovin (vincristine)
Prednisone
85
Q

Pt presents with superficial bleeding from skin and mucosal surfaces. Platelet count is normal. Bleeding gets worse every time they take aspirin. Dx?

A

Von willebrand dz

86
Q

Most accurate test for Von willebrand dz

A

Ristocetin cofactor assay and vWF level

87
Q

First line tx of Von willebrand dz

A

Desmopressin or DDAvp

- releases subendothelial stores of vWF and factor VIII

88
Q

Sudden appearance of rash and or bleeding in otherwise healthy child

A

Consider ITP

89
Q

What antibodies are present in ITP

A

Glycoproteins IIB/IIIa receptor abs

90
Q

Tx. Mild ITP (mild bleeding with platelets <50,000)

A

Prednisone

91
Q

Tx. ITP with platelet count <20,000 and serious bleeding (intracranial hemorrhage)

A

IVIG or Rho gam

92
Q

What drugs can be used in treatment of ITP if no response to splenectomy and still having recurrent episodes?

A

Romiplostim

Eltrombopag

93
Q

Tx. Uremia induced platelet dysfunction

A

Desmopressin

94
Q

Prolonged aPTT and bleeding that does not correct with a mixing study

A

Factor VIII antibody - MCC

95
Q

Pt who was given heparin three days ago for DVT prophylaxis comes in a new thrombosis - what should u be considering?

A

Heparin induced thrombocytopenia - drop in platelets few days after heparin treatment; thrombosis is most common clinical manifestation

96
Q

Best initial diagnostic test for HIT

A

Platelet factor 4 abs

Heparin induced anti platelet abs

97
Q

CCS - what physical exam stuff do you order for suspected anemia?

A

General appearance, cv, chest, extremities, HEENT

98
Q

Best initial therapy for HIT

A

Stop heparin

Give direct thrombin inhibitor (argatroban, lepirudin)

99
Q

Tx. HIV associated thrombocytopenia

A

Zidovudine
- min 600 mg/day
Corticosteroids will work but will recur after stopping them and can increase risk of opportunistic infections

100
Q

If an HIV positive patient adheres to a 3 drug HARRT regimen - when will their viral load decrease to < 50 copies/ml?

A

16-24 weeks

- measure viral load at 4 weeks, 8-12 weeks and then remeasure every 6-8 weeks thereafter

101
Q

tx. of inoperable head and neck cancer

A

combination chemo-radiotherapy

102
Q

Type 1 HIT

A

w/in 2d of initiation of heparin therapy; platelet count returns to normal with d/c of therapy

103
Q

Type 2 HIT

A

abs against heparin platelet factor 4 which causes platelet activation and aggregation leading to thrombocytopenia and platelet rich clots; usually 4-10 days after initiation of heparin therapy and can lead to arterial or venous thrombosis

104
Q

warfarin induced skin necrosis

A

high dose warfarin induces a transient hypercoagulable state by causing rapid reduction in protein C levels on the first day of therapy; lesions on breasts, trunk and extremities. No thrombocytopenia

105
Q

heparin induced skin necrosis

A

complication of unfractionated heparin; involves areas rich in fat

106
Q

Tx. in HIT

A

discontinuation of all heparin products and warfarin and start anticoagulation with direct thrombin inhibitors (lepirudin, argatroban)

107
Q

limitations of lepirudin and argatroban

A

lepirudin - used in caution in renal insuff pts

argatroban - use in caution with hepatic dysfunction

108
Q

MCC of anemia in pts with ESRD

A

EPO deficiency

109
Q

absolute iron deficiency

A

< 100 ng/ml

110
Q

iron supplementation in pts with ESRD is recommended when

A

when transferrin sat < 30% and ferritin < 500ng/ml

- iron supp. should be IV for pts receiving dialysis for ESRD

111
Q

pt with low Hb despite being on EPO, ferritin > 500 - next step/

A

increase dose of EPO

112
Q

tx. of anemia of chronic disease in patients with RA

A

anti-TNF agents, i.e. infliximab

113
Q

next step in eval of normocytic normochromic anemia

A

reticulocyte count

- to r/o hemolytic causes

114
Q

major problem leading to difficulty finding cross-matched blood in patients with a history of multiple transfusions

A

alloantibodies - most commonly, E, L and K

115
Q

indications for irradiated RBCs

A

BMT recipients
immunodeficiency pts
blood components donated by first or second degree relatives

116
Q

indications for leukoreduced RBCs

A

chronically transfused pts
CMV seronegative at risk pts (HIV, transplants)
potential transplant recipients
previous febrile non hemolytic transfusion reaction

117
Q

indications for washed RBCs

A

IgA deficiency
complement dep. autoimmune hemolytic anemia
continued allergic reactions with transfusion despite antihistamine treatment

118
Q

initial diagnostic test for pernicious anemia

A

anti-IF ab testing

119
Q

findings on biopsy in pernicious anemia

A

absent rugae in fundus and body–> autoimmune metaplastic atrophic gastritis

120
Q

EPO levels in polycythemia vera

A

normal or low

121
Q

polycythemia + high EPO levels

A

secondary causes

- consider getting nocturnal oximetry and carboxyhemoglobin

122
Q

tx. multiple brain mets

A

whole brain irradiation

- can improve survival 3-6 months

123
Q

screening while on tamoxifen

A

no additional screening needed

- annual gyn exam with complete history and routine pap smears

124
Q

1 unit of platelets should increase platelet count by how much

A

5000 platelets

- measure post transfusion count 10-60 minutes following transfusion

125
Q

platelet refractoriness

A 
absolute platelet count increment of less than or equal to 2000 / unit of platelets given
- MCC - alloimmunizatin (ab to HLA class 1 antigen on platelets)

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