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Step 3 > Hematology > Flashcards

Hematology Flashcards

1
Q

What are the “B symptoms” associated with Lymphoma?

A

Fever
Weight Loss
Night Sweats

(These indicate microscopic mets)

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2
Q

What is a common complication seen in patients with thalassemia major having recurrent transfusions?

A

Hemochromatosis

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3
Q

How is Thalassemia Major Transfusion-related Iron overload treated?

A

Deferroximine

Note: Phlebotomy would be counterproductive so cannot do it!

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4
Q

What is the next best step in managing a pt over 50yrs old with microcytic anemia and Negative Stool Guaiac and/or Negative Iron studies ?

A

Colonoscopy

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5
Q

What is the most common cause of Sideroblastic anemia?

A

Alcohol

Note: INH, Pb are other causes

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6
Q

Which Microcytic anemia is most likely to be associated with abnormal peripheral smear?

A

Thalassemia -target cells

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7
Q

Which type of microcytic anemia is most likely to have increased RDW?

A

Iron-deficiency Anemia

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8
Q

Which microcytic anemia is associated with low Ferritin?

A

Iron-deficiency Anemia

Note: Ferritin is also an acute phase reactant

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9
Q

What distinguishing test can be used to dx Fe-deficiency anemia?

A

TIBC-it will be High (b/c there are a lot of binding sites available for carrying iron when iron levels are low)

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10
Q

What is the most accurate test to dx iron-deficiency anemia?

A

Bone marrow Biopsy

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11
Q

Which microcytic anemia will have a high serum iron?

A

Sideroblastic Anemia

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12
Q

What is the most accurate test for sideroblastic anemia?

A

Prussian Blue Stain

see ringed-sideroblasts: iron deposits accumulated in mitochondria of RBC’s

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13
Q

T/F:

Iron studies are abnormal in all forms of thalassemia?

A

False:

All Thalassemias have normal iron studies.

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14
Q

What form of Thalassemia has a high Reticulocyte count?

A

Alpha thalassemia with 3 gene deletion. (Beta 4 tetrad HB formation)

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15
Q

What is the best initial test in patient with alcohol-induced macrocytic anemia?

A

Peripheral Smear (to check for hypersegmented neutrophils)

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16
Q

What is the most common neurological finding associated with B12 deficiency?

A

Peripheral Neuropathy

Note: Any/all types of neurological symptoms can manifest from B12 deficiency

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17
Q

What is the distinguishing feature of Folate deficiency anemia?

A

No neurological Symptoms/findings

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18
Q

What are some causes of Folate deficiency other than decreased intake?

A

Psoriasis (or other desquamating skin conditions)
Pregnancy
Sickle Cell Disease
All these represent very high cell turnover states

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19
Q

If you suspect B12 deficiency and pt has normal B12 levels what is next best step in management?

A

Methylmalonic Acid Level

It will be High in B12 deficiency but not folate deficiency

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20
Q

What metabolite is elevated in both folate and B12 deficiency?

A

Homocysteine

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21
Q

What is next best test once B12 or methylmalonic acid levels are determined?

A

Anti-Intrinsic Factor Ab’s and
Anti-Parietal Cell Ab’s

(these dx pernicious anemia)

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22
Q

What are common lab findings associated with Intravascular hemolysis?

A

Hemoglobinuria (toxic to cells)
Low Haptoglobin

Note: Unconjugated Bilirubin CANNOT filter through glomerular apparatus, it is INSOLUBLE. So dark urine is NOT due to Bbilirubin.

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23
Q

What are two renal complications of direct Hb exposure?

A

Renal Failure/Acute Tubular Necrosis (ATN)

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24
Q

What is the best initial test to dx sickle cell disease?

A

Peripheral Smear

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25
Q

What are the clinical manifestations of Sickle Cell trait?

A

Hematuria and Isosthenuria

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26
Q

What is the next best step in management in a pt with SCD with fever or elevated WBC?

A

Empiric Antibiotics

DO NOT WAIT FOR CULTURE RESULT

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27
Q

What is the etiology of a sudden drop in HCT in a pt with SCD?

A

Parvovirus B19 causing Aplastic Crisis

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28
Q

What is the next step in managing a pt. with SCD who develops a sudden drop in HCT?

A

Serum Parvovirus B19 PCR

Is PCR not available then do IgM anti-parvovirus Ab

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29
Q

What is the best treatment for SCD pt presenting with vision changes, CNS/stroke sx’s, acute chest syndrome, priaprism?

A

Exchange transfusion (Goal is to decrease HbS by 30-40%)

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30
Q

What preventative measures should be given to all patients with SCD?

A

Hydroxyurea
Folate replacement
Pneumococcal Vaccine

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31
Q

What drugs are associated with Autoimmune Hemolysis?

A

Penicillin
Alpha-methyldopa
Quinine/quinidine
Sulfa Drugs

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32
Q

What is the best initial treatment for Autoimmune Hemolysis?

A

Steroids (Prednisone)

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33
Q

What is the next best step in treatment for pt with Autoimmune Hemolysis presenting with recurrent hemolytic episodes?

A

Splenectomy

remove sight of extravascular RBC destruction. anti-RBC Ab’s will be present but the cells will not be destroyed

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34
Q

What two conditions are associated with spherocytosis?

A

Hereditary Spherocytosis

Autoimmune Hemolysis

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35
Q

What finding on CBC is associated with HS?

A

Elevated MCHC

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36
Q

What is the most accurate test for dx’ing HS?

A

Osmotic Fragility Test

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37
Q

What is the most accurate test for Paroxysmal Nocturnal Hemoglobinuria?

A

CD-55 and CD-59 antibody (serum)

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38
Q

What is best initial treatment for PNH?

A

Steroids

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39
Q

For transfusion dependent pts, what is best treatment?

A

Eculizumab (inhibits C-5, preventing complement activation)

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40
Q

What is the most likely condition associated with Hemolytic Anemia
Venous Thrombosis
Pancytopenia

A

Paroxysmal Nocturnal Hemoglobinuria (PNH)

41
Q

What test is the best initial test for dx’ing G6PD deficiency?

A

Heinz Body Test (Stain)

Bite Cell present on peripheral smear

42
Q

What is the most common cause of oxidative stress in G6PD deficiency?

A

Infection

43
Q

What is the most accurate test to Dx G6PD deficiency?

A

G6PD deficiency level

(but should only be done 2 months after presentation. At time of acute presentation, G6PD levels will be normal b/c deficient cells are destroyed first, leaving normal cells behind)

44
Q

What is the best treatment for HS?

A

Splenectomy

45
Q

What is an alternative treatment for Autoimmune Hemolysis and Cold Agglutinins?

A

Rituximab (anti-CD20 monoclonal Ab)

Note: It is also used to treat Rheumatoid Arthritis as one of the biologic DMARD’s

46
Q

How are G6PD def and Pyruvate Kinase Def distinguished?

A

Pyruvate Kinase def is not induced by oxidative stress.

47
Q

What test should be carried out prior to Dapsone use?

A

G6PD level or Heinz Bodies/Bite Cells

48
Q

What is the etiology of Aplastic Anemia?

A

Unknown cause of Killer T-cell induced Pancytopenia

49
Q

What is the treatment for Aplastic Anemia?

A

Cyclosporine and anti-Thymocyte Immunglobulin (horse-derived anti-Tcell Ab)

50
Q

What are typical symptoms of pancytopenia?(3)

A

Fatigue (decreased RBC’s)
Infection (WBC’s that do not degranulate)
Bleeding (low platelets)

51
Q

What is the most liklely dx for a pt presenting with anemia, thrombocytopenia, leukocytosis with blast predominance in DIC?

A

AML type M3

52
Q

What is the treatment for AML:M3

A

Daunorubicin+Cytosine Arabinoside+All Trans Retinoic Acid

53
Q

What is the treatment for ALL?

A

Daunorubicin+Vincristine+Prednisone +Intrathecal Methotrexate (for prophylaxis against spinal cord mets)

54
Q

What is used to determine who should undergo Bone Marrow Transplant immediately following remission induction?

A

Cytogenetics

Order Karyotype analysis as part of initial work up!

55
Q

What is the most likely dx in a pt presenting with Acute leukemia with a WBC ct >100,000, vision changes, shortness of breath, confusion?

A

Leukapharesis + Fluids

to rid body of large, excessive wbc’s–> treat leukostasis

56
Q

What is the treatment for Chronic Lymphocytic Leukemia (CLL)?

A

Fludarabine + Rituximab+ Cyclophosphamide

57
Q

What is an alternative treatment for CLL in pts who fail to respond to Fludarabine?

A

Alemtuzumab

58
Q

What is the mechanism of infection, anemia, and thrombocytopenia associated with late statge CLL?

A

CLL lymphocytes produce Abnormal/Insufficient Immunoglobulins –> RBC/Platelet attack by abnormal lymphocytes OR reduced amount of IgG –> Hemolysis/
thrombocytopenia and/or recurrent infection

59
Q

What test is used to disinguish between Chronic Myelogenous Leukemia (CML) and Leukemoid Reaction when pt presents with elevated WBC ct with neutrophil predominance?

A

Lekocyte Alkaline Phosphatase (LAP)

High: Leukemoid Rxn
Low: CML (a lot of neutrophils that don't work fully)
60
Q

What is the best initial test in work up of pt presenting with fatigue with or without enlarged lymph nodes?

A

CBC w/ differential:
if WBC high with >90% lymphocytes –> CLL

if WBC high with >90% Neutrophils –> Do LAP test

61
Q

What is the typical presentation of CML?

A

Fatigue
Early Satiety
Abdominal Pain
Splenomegaly

62
Q

What is standard treatment for CML in pt positive for Philadelphia Chromosome?

A

Imatinib (Gleevec) specifically targets the RTK BCR/ABL d/t Philadelphia chromosome. (But this is not a cure!!)

63
Q

What is the best treatment to cure CML?

A

Bone Marrow transplant (but this is not best initial treatment. Try Imatinibfirst.)

64
Q

What is most common presentation in pt with Multiple myeloma (MM)?

A

Bone Pain

d/t fracture with normal use

65
Q

What is next best step in pt with recent diagnosis of MM?

A

Skeletal Survey (look for lytic bone lesions)

Note only do bone scan if skeletal survey is inconclusive

66
Q

What tests should be ordered in workup of pt suspected of MM?

A 
Serum and Urine Protein Electrophoresis (BJP)
Skeletal Survey (Lytic Lesions)
BMP (Chem 7) (Hypercalcemia, BUN/Creat)
Peripheral Smear (Rouleaux)
Beta2 microglobulin (prognosis)
67
Q

What chemo is used to prepare for bone marrow transplant in the treatment of MM?

A

Adriamycin
Vincristine
Dexamethasone

68
Q

What is alternative treatment for pt with MM who is over 70 but relatively healthy?

A

Thalidomide (inhibits TNF) or Lenalidomide or

Bortezomib

69
Q

What other treatments are used for MM?

A
70
Q

What monoclonal Immunoglobulins are associated with MM?

A

Monoclonal IgG or IgA

71
Q

What immunoglobulins are associated with MGUS?

A

IgG (warm Agglutinins)

72
Q

What is the distinguishing features of Waldenstrom Macroglobulinemia-related hyperviscosity ?

A 
Blurry vision
Confusion
Headache
Shortness of Breath
IgM Spike on SPEP (cold agglutinins)
73
Q

What are the mechanisms of renal failure associated with MM?

A

Hypercalcemia: Nephrocalcinosis

Bence Jones Proteins: Clogs glomeruli and Toxic to tubules

Immunglobulins: clog glomerular apparatus

Hyperuricemia: Toxic to Tubules

Amyloid: Damage Nephron

74
Q

What type of bx should be done in a pt who presents with a neck mass that is firm, rubbery, non-tender, non-erythematous, not warm?

A

Excisional Biopsy

75
Q

What stages of Lymphoma can be treated with Radiation?

A

Stage I: single LN group involved
Stage II: multiple LN groups on same side of Diaphragm

(therefore, staging is next step in managing Lymphoma once diagnosed)

76
Q

What chemo therapy agents cause peripheral neuropathy?

A

Vincristine/Vinblastine

77
Q

Which Chemo agents cause Pulmonary Fibrosis?

A

Bleomycin/Blusulfan

78
Q

Which pts with Lymphoma are candidates for chemo?

A

Stage III and IV

Pt. with B symptoms

79
Q

What chemotherapy agent is cardiotoxic?

A

Adriamycin (daunarubicin)

80
Q

What tests should be done to work up a pt. suspected of Lymphoma?

A 
Excisional Bx
Chest Xray
CT (w/contrast) Head, Chest, Abdomen, Pelvis
Bone Marrow Bx
anti-CD20 Ag
81
Q

What is the treatment for Hodgkins Lymphoma?

A

Radiation

OR

Adriamycin
Bleomycin
Vincristine
Dexamethasone

82
Q

What hystologic finding is associated with Hodgkins Lymphoma?

A

Reed-Sternberg Cells

83
Q

What treatment is typically used to treat Non-Hodgkins Lymphoma?

A

CHOP and Rituximab (if anti-CD20 Ag present)

Cyclophosphamide (hemorrhagic cystitis)
Hydroxyadriamycin (cardiotoxicity)
Vincristine (peripheral neuropathy)
Prednisone (cushing syndrome, psychosis, bone pain/break down)

84
Q

What is next step in management in pt who presents with isolated thrombocytopenia, petechia, and epistaxis without a palpable spleen on exam?

A

Administer Prednisone

same as for autoimmune hemolysis with Coomb’s +

85
Q

If pt. with ITP on Predinose has recurrent episodes of bleeding/thrombocytopenia, what is best treatment ?

A

Splenectomy

Similar to autoimmune hemolysis

86
Q

What is the next step in a pt who presents with epistaxis, petichiae, melena, intraccranial hemorrhage, menorrhagia, (ie, life threatening bleeding) and platelet count

A

Administer IVIG or RhoGAM

(Fastest way to raise platelet count by occupying macrophages so they cannot destroy paltelets.
DO NOT GIVE PLATELETS.)

87
Q

What is the most likely dx in a young healthy female presenting with eoistaxis, petichiae, no spenomegaly, and isolated thrombocytopenia?

A

Idiopathic Thrombocytopenic Purpura (ITP)

88
Q

What are the distinguishing features associated with Factor XI deficiency?

A

Deep bleeding associated with rare trauma

Jewish

89
Q

What is a distinguishing feature associated with Factor XII deficiency?

A

No bleeding, no clotting

Prolonged PTT

90
Q

What is the most likely dx in a pt presenting with Renal failure, thrombocytopenia, and hemolysis following exposure to E. coli O157:87?

A

Hemolytic Uremic Syndrome

91
Q

What is the best initial treatment for pt. with HUS?

A

Mild: Symptomatic (self limiting)

If severe: Plasmapheresis

92
Q

What is the next best step in management for a pt presenting with Fever, anemia, thrombocytopenina, renal failure, and neurological findings?

A

Plasmapharesis (if severe)

93
Q

What is the most accurate test to determine VFW function?

A

Ristocetin test (platelets will stick together if VWF is working.

94
Q

What is the best initial therapy for Von Willebrand”s Disease?

A

DDAVP (Desmopressin)

95
Q

What other conditions can DDAVP be used to treat?

A

Uremic-induced Platelet Destruction

Mild Hemophelia A

96
Q

What is the treatment for severe Hemophelia A or B, or VWD?

A

Factor VIII replacement

This has both factor VIII and VWF, aka Factor VIII Ag.

97
Q

What coagulation study result is altered in a pt. with Lupus Anticoagulant?

A

Elevated PTT (that does not decrease)

98
Q

What is the most common cause of unprovoked thromboses with normal PTT?

A

Factor V Leiden Mutation

Resistant to Protein C

99
Q

What is the most likely dx in a pt with a low PTT that does not increase with heparin bolus?

A

Antithrombin III deficiency

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