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Hematology Flashcards

1
Q

Most common cancer of childhood
Immature B cells or T cells
Occurs before age 15 but most are age 1-3 yr
Cells: TdT+

A

Acute Lymphoblastic Leukemia

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2
Q

poor outcome in adult ALL

A

t(9;22) Philadelphia

Good outcome in child: t(12;21)

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3
Q

contains Major Basic Protein
Phagocytic for antigen-antibody complexes
Produces histaminase -> limits reaction following mast cell degranulation

A

Eosinophils

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4
Q

monocyte in the tissue

A

macrophage

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5
Q

mediates allergic reaction due to granule contents

heparin, histamine & leukotrienes

A

Basophil

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6
Q

mediates local allergic reaction
Binds Fc portion of IgE
Degranulates releasing histamine, heparin & eosinophilic chemotactic factors

A

Mast cell

Tx: cromolyn sodium

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7
Q

dendritic cell in skin

A

Langerhans cell

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8
Q

Functions as link between innate and adaptive immune systems.
Expresses Fc and MHC II
Highly phagocytic

A

dendritic cell

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9
Q

Where does B cell mature?

A

bone marrow

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10
Q

costimulatory signal required for T cell activation

A

CD28

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11
Q

chromatin distribution described as “clock-face”

A

plasma cell

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12
Q

Which antibodies cross the placenta?

A

IgG

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13
Q

Age: > 60 yr
CD20+, CD5+ B-cell neoplasm
smudge cells in peripheral smear
typically asymptomatic at diagnosis but may present with fatigue, anorexia, weight loss

A

Small lymphocytic lymphoma/chronic lymphocytic leukemia (CLL)

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14
Q

complication of CLL

A

hypogammaglobulinemia -> infection is most common cause of death

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15
Q 
Late adulthood painless lymphadenopathy
t(14;18)
BCL2 gene product overexpression -> inhibition of apoptosis
tumor arises in germinal centers
incurable with waxing & waning course
A

Follicular lymphoma

*note when any leukemia involves the lymph NODE it is referred to as LYMPHOMA

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16
Q

proliferation of large B cells that grow diffusely in sheets
high-grade
late adulthood
dysregulation of BCL6 (required for normal germinal center formation) -> p53 silenced -> no apoptosis

A

Diffuse Large B-cell Lymphoma

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17
Q 
tumor of mature B cells
extranodal mass in child or young adult
African form: mandible
Sporadic form: abdomen (ileocecum & peritoneum)
Translocation of c-MYC: t(8;14)
associated with EBV
starry sky
A

Burkitt lymphoma

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18
Q

Age: 65-70 yr
destructive plasma cell tumors involving axial skeleton
Most commonly involves: vertebral column, ribs, skull, pelvis, femur, clavicle and scapula
Lytic, punched out vertebral & skull lesions on xray-> fracture risk
free Ig light chain excretion in urine (Bence Jones proteinuria)
Hypercalcemia: confusion, weakness, lethargy, constipation, polyuria (renal dysfunction)

A

Multiple Myeloma
cause of death: infection
2nd most common cause of death: renal insufficiency
CRAB: hyperCalcemia, Renal insuff, Anemia, Bone lesions/Back pain

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19
Q

hyperviscosity syndrome caused by excess secretion of IgM

no lytic bone lesions

A

Waldenstrom macroglobulinemia

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20
Q

asymptomatic plasma cell dyscrasia

precursor to multiple myeloma (1-2%)

A

MGUS => monoclonal gammopathy of undetermined significance

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21
Q

proliferation of small B cells in males age 50s-60s
t(11:14)
cyclin D1 overexpression -> promotes G1/S transition -> proliferation
expansion of mantle zone
symptoms related to spleen and gut in 50%

A

Mantle Cell Lymphoma

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22
Q

Arise in tissues involved in chronic inflammatory disorders of infectious or autoimmune etiology (Hashimoto, Sjogren, H pylori gastritis)
Memory B cell origin
More common in southern Europe than US
Ex: MALToma

A

Marginal Zone Lymphoma

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23
Q

B cell neoplasm of middle-aged white males
TRAP
“dry tap” due to marrow fibrosis

A

Hairy Cell Leukemia
cells have hairy cytoplasmic processes
Tx: cladribine

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24
Q

T cell tumors that home to the skin
Early stages have eczema-like lesions
Cells can spread to the blood and cause Sezary syndrome -> lymphocytes with cerebriform nuclei

A

Mycosis Fungoides

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25
Q

-Endemic to southern Japan, West Africa and Caribbean basin
-associated with HTLV-1 -> encodes Tax which activates NF-kappaB
-Skin lesions (rash), generalized lymphadenopathy, hepatosplenomegaly, peripheral blood lymphocytosis and hypercalcemia
Rapidly fatal (months-1 yr)

A

Adult T cell Leukemia/Lymphoma

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26
Q

○ Large multimeric glycoprotein that stabilizes factor VIII and protects it from degradation
○ Synthesized by endothelial cells and megakaryocytes
○ Concentrated in the endothelium of blood vessels and released in response to stress hormones of endothelial damage

A

• Von Willebrand factor

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27
Q

extrinsic coagulation pathway vs. intrinsic coagulation pathway

A
  • Extrinisic pathway is initiated by trauma & tissue factor exposure
    • Intrinsic pathway is activated when factor XI is converted the factor XIa by thrombin
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28
Q

serine protease that degrades fibrin

A

plasminogen -> plasmin

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29
Q

vitamin K antagonist

A

warfarin

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30
Q

oxidation of Hb sulfhydryl groups
Denatured Hb precipitates
Seen with G6PD deficiency

A

Heinz bodies

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31
Q

Seen with functional asplenia -> normally removed

basophilic nuclear remnants

A

Howell-Jolly bodies

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32
Q

triad of iron deficiency anemia, esophageal webs and atrophic glossitis

A

Plummer-Vinson syndrome

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33
Q

homozygote with absent beta chain
marrow expansion causing “crew cut” on skull xray
chipmunk facies
extramedullary hematopoiesis
increased risk of parvo B19 aplastic crisis

A

beta-thalassemia major

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34
Q

deficiency of ADAMTS13 (which can be inherited or acquired)

decreased degradation of vWF multimers

A

Thrombotic Thrombocytopenic Purpura

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35
Q

Bleeding due to defective platelet aggregation (platelet plug) in response to ADP, collagen, epinephrine or thrombin because of deficiency or dysfunction of GpIIb-IIIa,
AR

A

Glanzmann Thrombasthenia

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36
Q

defect in platelet plug formation due to deficiency of GpIb

AR

A

Bernard-Soulier syndrome

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37
Q

antibodies to GpIIb/IIIa with splenic macrophage consumption of platelet/antibody complex
Bone marrow bx: increased megakaryocytes

A

Immune thrombocytopenia (ITP)

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38
Q

Hypercoagulability in whites with increased risk of cerebral vein thrombosis, especially with oral contraceptive use

A

Factor V Leiden

resists degradation by activated protein C

39
Q

inactivates factor V

A

protein C
PROC mutations causes deficiency
protein S enhances protein C cleavage of factor V

40
Q

X-linked intrinsic coagulation pathway defect
hemarthroses, easy bruising, increased PTT
classically affected male and carrier females

A

Hemophilia A: deficiency factor VIII
Hemophilia B: deficiency factor IX
cure: liver transplant

41
Q

see normal bleeding time but decreased synthesis of factors 2, 7, 9, 10, protein C & S

A

vitamin K deficiency

42
Q

which chromosome for ABO blood groups?

A

chromosome 9

43
Q

when is Rhogam given?

A

28-32 weeks

44
Q

inhibits ferrochelatase and ALA dehydratase therefore decreases heme synthesis
inhibits rRNA degradation -> basophilic stippling seen
symptoms: Burton lines on xray, encephalopathy, abdominal colic, sideroblastic anemia, wrist & foot drop

A

lead poisoning
ALA dehydratase contains zinc and is therefore sensitive to inhibition by lead
Tx: EDTA and dimercaprol; succimer in children

45
Q

glutamic acid -> valine change in codon for beta globin
AR hemolytic condition
painful swelling in hands and feet
splenic infarct at low O2 tensions

A

sickle cell anemia
HbS is less soluble so RBC less deformable when moving through capillaries
heterozygote advantage for malaria

46
Q

X-linked enzyme deficiency common in Mediterranean
hemolysis in response to bacterial & viral infections
Deficiency of reducing equivalents (NADPH) in RBC
drug-induced hemolysis

A

glucose 6 phosphate dehydrogenase deficiency
anemia with eating fava beans
Presents: back pain, hemoglobinuria a few days after oxidative stress
RBCs with Heinz bodies and bite cells on peripheral smear

47
Q

first-line therapy for CML

A

imatinib

MOA: inhibitor of BCR-ABL tyrosine kinase

48
Q

allosteric activator at low concentration, but is a competitive inhibitor for oxygen binding at higher concentration

A

carbon monoxide

49
Q

has 1 polypeptide chain and 1 O2 binding site
binds O2 better at low O2 concentrations
heart and skeletal muscle

A

myoglobin

50
Q

4 pyrrole rings linked by alpha-methylene bridges

A

heme

51
Q

competitive inhibitors of O2 binding

A

CO and NO

both bind to the O2 binding site on heme

52
Q

formed in RBCs from 1,3-bisphosphoglycerate &
binds to Hb in the central cavity formed by the 4 subunits and increases the energy required for conformational changes which facilitate O2 binding

A

2,3-BPG
levels of this increase at high altitudes in order to increase O2 delivery to the tissues
adjustment to high altitude occurs over approx lifespan of RBC

53
Q

bind to Hb and cause a conformational change which releases O2

A

protons

54
Q

What lab value predicts sickle cell crisis?

A

MCHC

55
Q

decreases cell volume by increasing potassium and water efflux
reduced chance of malaria development because of decreased lifespan of RBC (40 days for homozygote) & inhibition of cell lysis for parasitized RBCs

A

HbC
Crystals form in O2-rich blood, so formation is more likely in the larger vessels and less likely to cause vascular occlusion.
Crystallization only occurs with ligand bound

56
Q

hypersegmented neutrophils

A

folate and B12 deficiencies

57
Q

B12 deficiency causes

A

dietary, malabosorption, pernicious anemia, Diphyllobothrium latum (fish tapeworm), proton pump inhibitors

58
Q

hemoproteins

A 
hemoglobin
myoglobin
CYPs
catalase
myeloperoxidase
NO synthase
59
Q

cellular storage for iron

A

ferritin

60
Q

iron transport in the plasma

A

transferrin

61
Q

gene mutations which decrease hepcidin synthesis
chronic iron toxicity
increased iron absorption
iron deposition within the joints and tissues of the heart, liver and pancreas, causing organ damage and failure
slate-blue skin appearance

A

hemochromatosis

62
Q

rate-limiting enzyme in porphyrin synthesis in the liver

A

ALA synthase

63
Q

needed for hephaestin and ceruloplasmin activity (mobilization of iron)

A

copper

64
Q

defect in hydroxymethylbilane gene
accumulation of ALA and PMB precursors causes abdominal pain, neurologic dysfunction but NOT photosensitivity
Painful abdomen
Port wine urine
Polyneuropathy
Psychological disturbances
Precipitated by drugs, alcohol & starvation

A

Acute Intermittent Porphyria
AD
Tx: glucose and heme -> inhibit ALA synthase

65
Q

X-linked defect resulting in reduced catalytic activity of ALAS2
during the block in heme synthesis, iron delivery to the mitochondria continues so have accumulation of non-heme iron as ferrochelatase

A

sideroblastic anemia

66
Q

Glycoprotein produced by the kidney in proportion to intracellular oxygen concentration

A

erythropoietin

67
Q

○ Transfer of methyl group from N5-methyl-FH4 to homocysteine to form methionine
○ Rearrangement of methylmalonyl-CoA to form succinyl-CoA

A

functions of B12
if have deficiency see:
decrease in methionine and SAM
↑homocysteine in blood and methylmalonic acid in urine
Folate deficiency: ↑homocysteine but no methylmalonic acid in urine

68
Q

deficiency of intrinsic factor

A

pernicious anemia

69
Q

defect in UMP synthase causing inability to convert orotic acid to UMP (in the de novo pyrimidine synthesis pathway)
AR
megaloblastic anemia in children that cannot be cured with folate or B12

A

orotic aciduria

70
Q

oxidized free Hb

A

methemoglobin

71
Q

How do you test for folate deficiency?

A

If suspect folic acid deficiency, give large histidine load and patient will excrete FIGLU in urine
see bright red tongue too!

72
Q

proteins secreted by the salivary gland and gastric mucosa (parietal cells) & bind B12

A

haptocorrins

73
Q

RBC proteins

A

• Spectrin, ankyrin, anion exchange protein (band 3), band 4.1, band 4.2 and actin all participate in the cytoskeleton, giving the RBC its biconcave shape
Function of band 3: exchange of chloride and bicarbonate ions

74
Q

falsely elevated in anemia due to decrease in RBCs

A

reticulocyte count
a properly functioning marrow increases the RC to >3%
RC >3% suggests peripheral destruction

75
Q

RBC destruction by macrophages of spleen, liver & lymph nodes (RES)

A

extravascular hemolysis
see: anemia with splenomegaly, jaundice, increased risk of gallstones
spherocytes in peripheral smear

76
Q

hemolysis of RBCs within blood vessels

A

intravascular hemolysis
see: hemoglobinemia, hemoglobinuria, hemosiderinuria (iron), decreased serum haptoglobin
schistocytes and increased reticulocytes on peripheral smear

77
Q

AD: Mutations which deplete band 3, RhAG, band 4.2, ankyrin or spectrin
small round RBCs with no central pallor
increased RDW
premature removal of RBCs by the spleen

A

Hereditary spherocytosis

tx: splenectomy

78
Q

major energy source for RBC

A

glycolysis and pentose pathway shunt

79
Q

highly condensed X chromosome

A

Barr body

80
Q

Why does malaria grow poorly in G6PD deficiency?

A

ROS generation (glutathione depletion)

81
Q

phenotypically “O” blood type but also have anti-H antibody

A

Bombay blood type

82
Q

sugars that determine blood type

A

A: galactosamine
B: galactose

83
Q

> 20% blasts in the bone marrow

A

leukemia
will also see decreases in normal blood cells (RBCs -> anemia; neutropenia; thrombocytopenia)
Blasts in the blood are larger than normal RBCs and have large nucleus, little cytoplasm, punched-out nucleolus

84
Q

markers for myeloblasts vs lymphoblasts

A

Myeloblasts (AML): MPO -> look for Auer Rod**

Lymphoblasts (ALL): tDt

85
Q

accumulation of myeloblasts in AML has what major risk

A

DIC
AML is a disruption to the Retinoic Acid Receptor (RAR)
Treat with ATRA -> causes myeloblasts to mature to neutrophils

86
Q

subtype of myeloblastic leukemia in which presentation is swelling of the gums (blasts invade gums)
cells lack MPO

A

acute monocytic leukemia

87
Q

causes of myeloblastic syndrome

A

prior exposure to alkylating agent or radiotherapy

88
Q

neoplastic proliferation of naive B cells in older patient
coexistence of CD5 and CD20 is characteristic (CD5 is normally on T cells)
smudge cells

A

CLL

89
Q

Why is there an increased risk of hyperuricemia & gout in myeloproliferative disorders?

A

high cell turnover

90
Q

worst complication of CML

A 
acute leukemia (can convert to ALL or AML)
CML has a characteristic increase in basophils**
t(9;22) generating BCR-ABL fusion protein with increased tyrosine kinase activity
91
Q

proliferation of mature myeloid cells esp RBCs
Granulocytes & platelets also elevated
associated with JAK2 kinase mutation**
Symps: blurry vision, headache, flushed face (plethora), ITCHING after bathing (histamine release from extra mast cells), increased risk of venous thrombosis

A

Polycythemia Vera (PV)
Tx: phlebotomy (1st)
hydroxyurea (2nd)

92
Q

causes of reactive polycythemia

A

hypoxia from lung disease

EPO production by renal cell carcinoma

93
Q

neoplastic proliferation of megakaryocytes
assoc with JAK2 kinase mutation
progresses to marrow fibrosis
Slide: tear-drop RBCs, nucleated RBCs, immature granulocytes
Increased risk of infection, thrombosis, bleeding

A

Myelofibrosis

complications: splenomegaly from extramedullary hematopoiesis

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