Hematology Flashcards

1
Q

Best initial test for anemia?

A

CBC with peripheral smear

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2
Q

What are the other initial tests for anemia?

A

Reticulocyte count, haptoglobin, LDH, total bilirubin, TSH with T4, B12/folate, iron studies

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3
Q

What is the best initial diagnostic test for iron deficiency anemia?

A

Iron studies-

  • Low ferritin
  • High TIBC
  • Low Fe
  • Low Fe sat
  • Elevated RDW
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4
Q

What is the best initial therapy for iron deficiency anemia?

A

ferrous sulfate orally

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5
Q

What is the presentation of patients with anemia of chronic disease?

A

patients with RA, ESRD, any chronic disease

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6
Q

What is the best initial diagnostic test for anemia of chronic disease?

A

Iron Studies-

  • High ferritin
  • Low TIBC
  • Low Fe
  • Normal/ Fe sat
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7
Q

What is the best initial therapy for anemia of chronic disease?

A

Correct underlying disease

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8
Q

How does sideroblastic anemia present?

A

alcoholism, isoniazid use, lead exposure

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9
Q

What is the best initial test for sideroblastic anemia?

A

Iron studies- shows high Fe

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10
Q

What is the most accurate test for sideroblastic anemia?

A

Prussian blue stain

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11
Q

What is the best initial therapy for sideroblastic anemia?

A

Remove the toxin exposure, possibly pyridoxine

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12
Q

How does Vitamin B12 deficiency present?

A

Macrocytic anemia, neurologic findings(peripheral neuropathy), smooth tongue

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13
Q

How does folate deficiency present?

A

Same as B12 deficiency without the neurologic problems

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14
Q

What is the best test for macrocytic anemias?

A

CBC with peripheral blood smear(look for hypersegmented (>5 segments) neutrophils)

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15
Q

What else should be ordered in the CCS portion for megaloblastic anemia?

A

Biliubin and LDH levels- elevated

Reticulocyte- low

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16
Q

What is the most accurate test for megaloblastic anemia?

A

Low B12(for B12 deficiency) and folate levels(for folate deficiency)

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17
Q

What is the next best test to confirm B12 deficiency etiology after finding a low B12 or elevated methylmalonic acid ?

A

Anti-parietal cell antibodies and anti-intrinsic factor antibodies

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18
Q

What is the treatment for B12 and folate deficiencies?

A

replacement

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19
Q

What is seen on the labs for hemolytic anemia with regards to the following?
Indirect bilirubin, reticulocyte count, LDH, haptoglobin

A

Indirect bilirubin- elevated
reticulocyte count- elevated
LDH- elevated
haptoglobin- decreased

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20
Q

In cases of hemolysis on the CCS exam, what should you order?

A

Peripheral smear, LDH, bilirubin level, reticulocyte count, haptoglobin

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21
Q

What must you do when there is a patient with sickle cell disease?

A

complete physical exam

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22
Q

What is the next best step when a diagnosis of sickle cell disease is made?

A

Give oxygen, hydration, and pain meds

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23
Q

If fever is present with sickle cell disease, what is the next step?

A

give ceftriaxone, levofloxacin, or moxifloxacin

fever is an emergency because they functionally have no spleen

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24
Q

When is exchange transfusion the answer for a patient with sickle cell disease?

A
  • when having visual changes from retinal infarction
  • pulmonary infarction leading to pleuritic pain
  • priapism
  • stroke
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25
Q

If a sickle cell patient has a sudden drop in Hct, what is the diagnosis?

A

Parvovirus B19 or folate deficiency

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26
Q

What is the diagnostic test for autoimmune hemolysis?

A

LDH, Indirect bilirubin, reticulocyte levels will be increased. haptoglobin will be decreased

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27
Q

What is the most accurate test for hemolysis?

A

Coombs test

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28
Q

What is the best initial therapy for hemolysis?

A

steroids; if case describes recurrent hemolysis, splenectomy is the most effective therapy

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29
Q

If severe hemolysis is unresponsive to repeated tranfusions and prednisone, what is the next step?

A

IV IgG

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30
Q

What history do patients with cold induced hemolysis have?

A

History of mycoplasma or EBV

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31
Q

What are the diagnostic tests for cold induced hemolysis?

A

Coombs positive. complement test positive

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32
Q

What is the treatment for cold induced hemolysis?

A

If no response to steroids, splenectomy, or IVIG…..

Treat with rituximab

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33
Q

How does G6PD Deficiency present?

A

sudden onset of hemolysis, X-linked, males, most commonly associated with ongoing infection, oxidizing drugs(sulfa, primaquine)

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34
Q

What is the best initial test for G6PDD?

A

Heinz body test, bite cells

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35
Q

What is the most accurate test for G6PDD?

A

G6PD level(only test after 2 months have passed from initial attack)

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36
Q

What is the treatment for G6PDD?

A

Avoid oxidant stress

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37
Q

How does hereditary spherocytosis present?

A

Recurrent hemolysis, splenomegaly, gallstones, increased MCHC

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38
Q

How do you diagnose hereditary spherocytosis?

A

osmotic fragility test(most accurate)

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39
Q

What is the treatment for hereditary spherocytosis?

A

splenectomy

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40
Q

What are HUS/TTP associated with?

A

E. coli 0157:H7 infection with HUS, ticlopidine is associated with TTP

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41
Q

What is the HUS triad?

A

ART
Autoimmune hemolysis (intravasular hemolysis)
Renal failure (Elevate BUN and creatinine)
Thrombocytopenia

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42
Q

What is the TTP pentad?

A
FAT RN
Fever
Autoimmune hemolysis
Thrombocytopenia
Renal Failure
Neuro abnormalitites
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43
Q

What is the treatment for severe TTP or HUS?

A

Plasmapheresis; most cases resolve on their own. DO NOT GIVE PLATELETS

44
Q

How does paroxysmal nocturnal hemoglobinuria present?

A

Recurrent episodes of dark urine in the morning, pancytopenia. Most common cause of death is large vessel venous thrombosis

45
Q

What is the most accurate test for paroxysmal nocturnal hemoglobinuria?

A

CD55 and CD59 (aka decay accelerating factor)

46
Q

What is the best initial treatment for paroxsymal nocturnal hemoglobinuria?

A

prednisone

47
Q

How does methemoglobin present?

A

SOB for no clear reason, clear lung exam, normal CXR.
Hgb is locked in oxidized state and it can not pick up oxygen. Look for exposure to drugs(nitroglycerin, amyl nitrate, any -caine

48
Q

What are the characteristics of Transfusion-related acute lung injury(TRALI)?

A

SOB from antibodies in donor blood against recipient white cells. No treatment, resolves spontaneously

49
Q

What are the characteristics of IgA deficiency with transfusions?

A

IgA deficiency presents as anaphylaxis

50
Q

What are the characteristics of ABO incompatibility?

A

Acute symptoms of hemolysis (hypotensive, tachycardic, SOB) Normal LDH and bilirubin levels

51
Q

What are the characteristics of minor blood group incompatibility?

A

presents with delayed jaundice

52
Q

What is the best initial test for acute leukemia?

A

Peripheral smear showing blastic cells

53
Q

What is the best initial treatment for acute myelogenous leukemia?

A

Chemotherapy with idarubicin and cytosine arabinoside

54
Q

What is the best initial treatment for Acute Promyelocytic Leukemia (M3)?

A

Idarubicin + cytosine arabinoside + ALL TRANS RETINOIC ACID

55
Q

What is the best initial treatment for ALL?

A

Idarubicin + cytosine arabinoside + INTRATHECAL METHOTREXATE

56
Q

What condition is associated with Acute promyelocytic leukemia(M3)?

A

DIC

57
Q

How does myelodysplasia present?

A

Elderly patient, pancytopenia, Elevated MCV, low reticulocyte count, Neutrophils(2 lobed) “Pelger-Huet” cells. Normal B12

58
Q

What is the treatment for myelodysplasia?

A

Transfusions

59
Q

How does CML present?

A

elevated WBC, splenomegaly

60
Q

What is the best initial test for CML?

A

Elevated neutrophils with LOW LAP score(increased with infection)

61
Q

What is the most accurate test for CML?

A

Philadelphia chromosome (PCR) or BCR/ABL by FISH

62
Q

What is the best initial therapy for CML?

A

Imatinib (Gleevac). Bone marrow transplantation is curative, but never the best initial therapy

63
Q

How does CLL present?

A

patients >50 yo. Elevated WBC with “normal appearing lymphocytes”

64
Q

What is the best initial test for CLL?

A

Peripheral blood smear shows smudge cells(ruptured nuclei of lymphocytes)

65
Q

What are the stages of CLL?

A
Stage 0: Elevated WBC count
Stage 1: Enlarged lymph nodes
Stage 2: Spleen enlargement
Stage 3: Anemia
Stage 4: Low platelets
66
Q

What is the treatment for CLL?

A

Based on stage- No treatment for Stage 0,1.

More advanced stages are treated with fludarabine with rituximab

67
Q

How does hairy cell leukemia present?

A

Patient in mid 50s, pancytopenia, massive splenomegaly

68
Q

What is the most accurate test for Hairy cell leukemia?

A

Smear showing hairy cells and immunophenotyping, TRAP+

69
Q

What is the best initial therapy for hairy cell leukemia?

A

Cladribine

70
Q

How does polycythemia vera present?

A

Headaches, blurred vision, pruritis usually after a hot bath/shower, splenomegaly is common

71
Q

What is the key to diagnosis for polycythemia vera?

A

High HCT in absence of hypoxia, with low MCV. EPO is low

72
Q

What should be ordered during CCS portion with Polycythemia vera?

A

EPO level, which should be low, Hem/Onc consult, nuclear red cell mass test

73
Q

What is the best initial treatment for polycythemia vera?

A

phlebotomy
hydroxyurea is also given to lower cell count
daily aspirin should also be given

74
Q

What is the presentation of multiple myeloma?

A

bone pain caused by a fracture

75
Q

What are the initial tests for Multiple Myeloma?

A

skeletal survey
SPEP (elevated levels of IgG)
UPEP (elevated Bence-Jones)
Peripheral smear (shows rouleaux)
Beta-2-microglobulin (this is prognostic)
BUN/Creatinine (detects occurrence of renal insufficiency)

76
Q

What is the most specific test for multiple myeloma?

A

bone marrow biopsy

77
Q

What is the treatment for multiple myeloma?

A

Mephalan and steroids; thalidomide, lenalidomide, or bortezomib may be added

78
Q

What is the most effective treatment for multiple myeloma?

A

bone marrow transplant

79
Q

How does monoclonal gammopathy of unknown significance(MGUS) present?

A

asymptomatic elevation of IgG on SPEP.

No treatment needed

80
Q

How does Waldenstrom’s Macroglobulinemia present?

A

hyperviscosity from IgM overproduction, presents blurry vision, confusion, headache

81
Q

What is the best initial test for Waldenstrom’s Macroglobulinemia?

A

Serum viscosity level which is elevated

82
Q

What is the best initial treatment for Waldenstrom’s Macroglobulinemia?

A

plasmapheresis

83
Q

What is the best therapy for aplastic anemia?

A

BM transplant; if BM transplant not possible, antithymocyte globulin and cyclosporine

84
Q

How does lymphoma present?

A

enlarged lymph nodes around the neck

85
Q

How does Hodgkin’s disease present?

A

Spreads centrifugally away from the neck

86
Q

How does Non-Hodgkin’s Lymphoma present?

A

presents as widespread disease, fever, weight loss, night sweats

87
Q

What is the best initial test for diagnosis of lymphoma (HD or NHL)?

A

excisional lymph node biopsy; do not answer needle biopsy

88
Q

What does HD have that NFL doesn’t have?

A

HD has Reed-Sternberg cells

89
Q

What is the next step after the excisional biopsy?

A

Stage the disease
Stage I- single lymph node
Stage II- 2 lymph node groups on one side of the diaphragm
Stage III- nodes on both sides of diaphragm
Stage IV- widespread disease

90
Q

How is staging done with lymphoma?

A

CXR, CT scan(chest, abdomen, pelvis), bone marrow biopsy

91
Q

What is the treatment for lymphoma?

A
Localized disease(Stages I&II)- treated with radiation and low-dose chemo
More advanced(Stages III&IV)- treat exclusively with chemo
92
Q

What is the specific treatment regimen for Hodgkin’s lymphoma?

A
ABVD-
Adriamycin
Bleomycin
Vinblastine
Dacarbazine
93
Q

What is the specific treatment regimen for Non-Hodgkin’s Lymphoma?

A
CHOP-
Cyclophosphamide
Hydroxyadriamycin
Oncovin
Prednisone
Also, test for anti-CD20 antigen, if present add rituximab
94
Q

How does von Willebrand’s disease present?

A

bleeding from platelet dysfunction (epitaxis, bleeding from skin/mucosal surfaces…)

95
Q

What is the diagnostic test for von Willebrand’s disease?

A

ristocetin cofactor assay and vWF level

96
Q

What is the first line treatment for von Willenbrand’s disease?

A

desmopressin or DDAVP; if DDAVP is not effective, next step is to use factor VIII replacement

97
Q

How does ITP present?

A

platelet type bleeding(petechiae, epitaxis, purpura, gingival bleeding); platelet type bleeding < 10,000-30,000

98
Q

What is the diagnostic test for ITP?

A

peripheral smear shows large platelets, sonogram to assess for normal spleen size found in ITP, antibodies to glycoprotein IIb/IIIa receptor

99
Q

What is the first step in therapy for mild ITP?

A

prednisone- starting treatment is more important than finding diagnosis

100
Q

When a patient with ITP presents with a platelet count of <20,000, what is the medication that needs to be given?

A

IVIG, this will usually present as bleeding into brain or bowel

101
Q

Summary of treatment of ITP based on platelet count…..

A

> 50,000- No treatment
<20,000 with major bleeding- IVIG
Recurrent episiodes- splenectomy
No response to splenectomy- romiplostim, eltrombopag

102
Q

What is the presentation of factor 8 deficiency?

A

Joint bleeding/hematoma in male child

103
Q

What is the diagnostic test for factor 8 deficiency?

A

mixing study first, then specific factor

104
Q

What is the treatment for factor 8 deficiency?

A

Severe deficiency- Factor 8 replacement

Minor deficiency- DDAVP

105
Q

How does heparin induced thrombocytopenia present?

A

drop in platelets (50%) a few days after starting heparin with thrombosis as most common presentation

106
Q

What is the best initial diagnostic test for HIT?

A

Platelet Factor 4 antibodies or heparin induced antiplatelet antibodies

107
Q

What is the best initial therapy for HIT?

A

stop the heparin and use direct thrombin inhibitor