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CM 4 > Heme Diagnosis > Flashcards

Heme Diagnosis Flashcards

1
Q

immune thrombocytoenic purpura

A

CBC and smear- normal

Bone marrow aspiration - megakaryocytes

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2
Q

heparin induced thrombocytopenia

A

Thrombocytopenia, thrombosis, and timing of platelet drop.

HIT antibody testing.

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3
Q

disseminated intravascular coagulation (DIC)

A

Increased thrombin formation –> decreased fibrinogen.
Bleeding –> increased pt, ptt, inr; thrombocytopenia.
Increased fibrinolysis –> increased d-dimer.
Peripheral smear –> fragment rbcs, schistocytes.

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4
Q

hemophilia a and b

A

PTT prolonged.
Normal PT.
Platelets normal.

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5
Q

von willebrand disease

A

aPTT (to check factor VIII activity) - usually prolonged
vWF antigen test - decreased vWF antigen or activity
vWF activity (Ristocetin cofactor assay) - in VWD, no platelet aggregation will occur
Factor VIII activity - may be decreased

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6
Q

factor v leiden mutation

A

activated protein c resistance assay and if + confirm with dna testing.
Nml pt and ptt.

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7
Q

protein c or s deficiency

A

functional assay of protein c and s

plasma protein and c and s antigen levels

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8
Q

hemolytic anemias

A

peripheral smear: spherocytes, schistocytes, increased reticulocytes

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9
Q

sickle cell trait

A

electrophoresis: Presence of hemogloblin A and Hemoglobin S

blood counts and peripheral smear: normal

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10
Q

sickle cell disease

A

Counts -> decreased hemoglobin and/or hematocrit when in crisis.
Peripheral smear -> sickled cells, target cells, Howell-Jolly bodies (indicates functional asplenia)
Electrophoresis -> HbS present, little to no HbA, increased HbF
DNA analysis is the definitive test for diagnosis

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11
Q

alpha thalassemia- aka hemoglobin h disease

A

RBC count = increased
RBC shape = microcytic
RBC color = hypochromic
MCHC = low
RBC shape = Heinz bodies, schistocytes, target cells, tear drop cells
Reticulocytes = increased
Electrophoresis = presence of beta chain tetramer (HbH)
Iron = normal or increased serum iron due to iron overload

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12
Q

beta thalassemia major (Cooley’s Anemia)

A

Skull x-rays
Frontal bossing
“Hair on end” appearance of the skull

Blood
RBC count = normal or increased
RBC shape = microcytic
RBC color = hypochromic
MCHC = low
RBC shape = target cells, tear drop cells
Reticulocytes = decreased
Electrophoresis = Increased HgbF and HgbA2, little to NO HgbA
Iron = normal or increased serum iron due to iron overload

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13
Q

G6PD deficiency

A

Normocytic hemolytic anemia during crises.
Peripheral smear = during episodes, schistocytes (or bite cells), Heinz bodies is hallmark
Increased reticulocytes, increased bilirubin, decreased haptoglobin.
Enzyme assay for G6PD
DNA testing

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14
Q

Hereditary spherocytosis (HS)

A

Blood counts = microcytic, hyperchromic (increased MCHC)
Smear = spherocytes, possible schistocytes, increased reticulocytes
EMA Binding = preferred test (most accurate)-Flow cytometric analysis of eosin-5’-maleimide-labeled intact red blood cells & acidified glycerol lysis test
Osmotic fragility test - RBCs placed in a relatively hypotonic solution rupture easily due to the increased permeability of the RBC membrane
Negative Coombs testing

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15
Q

autoimmune hemolytic anemia

A 
Decreased Hgb
Increased reticulocytes
Increased MCHC
Peripheral smear = polychromasia, microspherocytes
POSITIVE DIRECT COOMB test
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16
Q

thrombotic thrombocytopenia purpura (TTP)

A

Thrombocytopenia with normal coagulation studies
Peripheral smear = hemolysis -> schistocytes, bite or fragmented cells, reticulocytes
Increased LDH & bilirubin
Decreased haptoglobin (protein that the body uses to clear free hemoglobin from circulation)
Decreased ADAMTS13 levels
Coombs negative

17
Q

Hemolytic uremic syndrome (HUS)

A

Labs the same as in TTP
Thrombocytopenia with normal coagulation studies
Peripheral smear = hemolysis -> schistocytes, bite or fragmented cells, reticulocytes
Increased LDH & bilirubin
Decreased haptoglobin
Increased BUN & creatinine
Coombs negative

18
Q

Paroxysmal Nocturnal Hemoglobinuria

A 
Hemoglobinuria
Increased reticulocytes
Increased bilirubin
Flow cytometry to look for CD55-CD59-deficient RBCs
Coombs negative
19
Q

iron deficiency anemia physical exam

A 
Koilonychia (nail spooning)
Angular cheilitis
Tachycardia
Glossitis
Pallor
20
Q

iron deficiency anemia

A 
Counts = microcytic (low MCV), hypochromic (low heme)
Smear = decreased reticulocytes
Iron Studies: 
Ferritin = decreased (pathognomonic)
TIBC = increased
Transferrin saturation = < 20-15%
Serum Iron = decreased
21
Q

lead poisoning

A

Serum lead levels: > 10 mcg/dL (venous sampling more accurate than finger stick)
Peripheral smear: microcytic, hypochromic anemia with basophilic stippling
Bone marrow: ringed sideroblasts

Iron Studies:
Serum iron: normal or increased
TIBC: decreased

22
Q

anemia of chronic disease

A 
Counts = Hgb around 9-10 mg/dL; RDW = normal to increased
Smear = normocytic, normochromic, decreased reticulocytes -> will eventually become microcytic
Iron Studies:  
Ferritin normal to increased
TIBC decreased
Serum iron decreased 
Hepcidin increased
23
Q

aplastic anemia

A

CBC = at least two cytopenias
Smear = few or absent reticulocytes
Bone marrow biopsy = most accurate test -> hypocellular, fatty bone marrow

24
Q

b12 deficiency

A

Counts = MCV increased (macrocytic)
Smear =
Megaloblastic anemia = hypersegmented neutrophils, macro-ovalocytes, mild leukopenia and/or thrombocytopenia
Reticulocytes decreased

Serum B12 decreased
Homocysteine increased
LDH increased
Methylmalonic acid increased (differentiates it from folate deficiency)

25
Q

folate deficiency

A 
Counts = MCV increased (macrocytic)
Smear = megaloblastic anemia
Reticulocytes = decreased
Serum folate = decreased
Homocysteine = increased
Methylmalonic acid = normal (B12 deficiency -> increased)
26
Q

hereditary hemochromatosis

A

Iron studies:
Serum iron, ferritin & transferrin saturation = increased
TIBC normal or decreased
Genetic testing for HFE gene
Liver biopsy = most accurate test -> increased hemosiderin

27
Q

polycythemia vera (primary erythrocytosis) PE

A

Hepatosplenomegaly
Facial plethora (flushed face)
Engorged retinal veins

28
Q

polycythemia vera (primary erythrocytosis)

A

All three major indicators OR two major and 1 minor:

Major
Increased RBC mass (increased Hgb & Hct [54% men; 51% women]).
Bone marrow biopsy with hypercellularity (extra erythroid, granulocytic & megakaryocyte cells).
JAK2 mutation.

Minor
Decreased serum erythropoietin levels.
Increased leukocyte alkaline phosphatase.
Increased granulocytic WBCs, platelets or B12.
Iron deficiency.

29
Q

myelodysplastic syndrome

A

CBC with peripheral smear = decrease in one or more myeloid cell lines (platelets, neutrophils or RBCs); hypo-segmented neutrophils, normocytic or macrocytic anemia

Bone marrow biopsy =
Normal or hypocellular (20% associated with hypocellularity)
Dysplastic bone marrow is the HALLMARK - increased myeloblasts but < 20%, ringed sideroblasts, pseudo Pelger-Huet cells (hypo-segmented and hypo-granulated neutrophils)

30
Q

acute myeloid leukemia (aml)

A

GOLD STANDARD = bone marrow biopsy -> AUER rods and >20% myeloblasts
BEST INITIAL TEST = CBC with peripheral smear -> normocytic, normochromic anemia with normal or decreased reticulocyte count, thrombocytopenia and possible circulating myeloblasts.
Immunophenotyping/FISH analysis = most accurate test -> myeloperoxidase positive

31
Q

chronic myelogenous leukemia

A

CBC with peripheral smear = leukocytosis with granulocytic cells (neutrophilia, basophilia, eosinophilia).
Leukocyte alkaline phosphatase score = decreased

Bone marrow biopsy = granulocytic hyperplasia:
Chronic -> < 5% blasts
Accelerated = 5-30% blasts
Acute blast crisis = > 20% blasts

Immunophenotyping/FISH analysis = Philadelphia chromosome

32
Q

acute lymphocytic leukemia (all) PE

A

Hepatomegaly or splenomegaly most common finding, which can manifest as anorexia, weight loss, abdominal distention or abdominal pain.
Lymphadenopathy

33
Q

acute lymphocytic leukemia (all)

A

CBC and peripheral smear = WBCs 5000-100,000, anemia, thrombocytopenia
Bone marrow aspiration = hypercellular with >20% blasts (definitive diagnosis)

34
Q

chronic lymphocytic leukemia (cll) PE

A 
Lymphadenopathy = 
Most common finding (cervical, supraclavicular axillary); LN are usually firm, round, nontender and freely mobile.
Splenomegaly  = painless and nontender
Hepatomegaly
Skin lesions (leukemia cutis)
35
Q

chronic lymphocytic leukemia (cll)

A

CBC with peripheral smear = absolute lymphocytes > 5,000/microL; small, well-differentiated normal-appearing lymphocytes with scattered smudge cells (lab artifact when the fragile B cells become crushed by the cover slip during slide prep); neutropenia

Hypogammaglobulinemia -> increased incidence of autoimmune hemolytic anemia; may have evidence of ITP.

Immunophenotypic analysis = expression of B-cell associated antigens (CD19, CD 20, CD 23) and B-cell maturity (CD5).

Bone marrow aspiration not needed

36
Q

multiple myeloma

A

Serum protein electrophoresis = monoclonal protein spike = IgG most common (60%)
Urine protein electrophoresis = Bence-Jones proteins
CBC and peripheral smear = ROULEAUX formations; increased ESR
Skull radiographs = “punched out” lytic lesions
Bone marrow aspiration= plasmacytosis > 10% = definitive diagnosis

37
Q

hodgkin lymphoma

A

Excisional whole lymph node biopsy = Reed Sternberg cell pathognomonic (cells with “owl eye appearance”).
Imaging (PET/CT scan) for staging.

38
Q

non hodgkin lymphoma

A

Lymph node and/or tissue biopsy

Staging via PET/CT scan

39
Q

labs for tumor lysis syndrome

A

Hyperphosphatemia, hypocalcemia, hyperuricemia, hyperkalemia and acute kidney injury

CM 4 (9 decks)

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