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Test reviews DEDICATED > hematology > Flashcards

hematology Flashcards

1
Q

pathophys of immune thrombocytponeic purpura?

A

autoimmune production of IgG against platelet antigens, ->consumed by splenic macrophages -> thrombocytopenia

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2
Q

which cells produce the antibodies in ITP?

A

splenic plasma cells

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3
Q

Lab findings in ITP

A

Decreased platelet count
Normal PT/PTT
Increased megakaryocytes on bone marrow biopsy

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4
Q

Treatment for ITP?

A

Corticosteroids, IVIG

-splenectomy for refractory cases - eliminates the site of the antibody production

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5
Q

Name two types of microangiopathic hemolytic anemia

A
  • HUS

- TTP

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6
Q

thrombotic thrombocytopenic purpura pathophs (TTP)?

A

Decreased ADAMTS13 - ADAMST13 normally cleaves vWF multimers into smaller monomers for degradation. With decreased ADAMTS13, uncleaved monomers lead ot abnormal platelet adhesion - > microthrombi -> sheering of rbcs

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7
Q

Decreased ADAMTS13 is usually due to…

A

an acquired autoantibody

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8
Q

who usually gets HUS?

A

children with e coli O157:H7 infection

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9
Q

Clinical findings of microangiopathic hemolytic anemia (HUS and TTP)?

A
  • skin and mucosal bleeding
  • hemolytic anemia
  • fever
  • renal insufficiency (HUS)
  • CNS abnormalities (TTP)
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10
Q

lab findings of microangiopathic hemolytic anemia (HUS and TTP)?

A
  • thrombocytopenia with increased bleeding time
  • normal PT/PTT
  • anemia with schitocytes
  • increased megakaryocytes on biopsy
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11
Q

uremia disrupts which parts of platelet function?

A

both adhesion and aggregation

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12
Q

bernard-soulier syndrome is due to a genetic deficiency of…

A

GPIb

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13
Q

what is the cause of glanzmann thrombasthenia?

A

genetic GPIIb/IIIa deficiency

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14
Q

Hemophilia A is a X linked deficiency of…

A

factor VIII

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15
Q

Hemophilia B =

A

Christmas Disease

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16
Q

Hemophilia B is a deficiency of…

A

factor IX

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17
Q

What is the most common coagulation factor inhibitor?

A

anti-FVIII

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18
Q

How can you differentiate hemophilia A from a coagulation factor inhibitor (autoantibody against factor VIII)

A

Upon mixing the patients plasma with normal plasma, the PTT will not correct due to the presence of the antibody (whereas it would correct with hemophilia A)

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19
Q

Most commonly inherited coag disorder?

A

Von Willebrand disease- genetic vWF deficiency

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20
Q

inheritance of most common type of vWF defieincy?

A

AD

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21
Q

lab findings of vWF disease?

A
  • increased bleeding time
  • increased PTT, normal PT
  • abnormal ristocetin test
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22
Q

why is PTT increased in vWF disease?

A

VWF normally stabilizes factor VIII

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23
Q

treatment of vWF disease?

A

desmopressin

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24
Q

function of alpha 2 antiplasmin?

A

inactivates plasmin

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25
Q

why may an alcohol have decreased alpha 2 antiplasmin?

A

liver cirhossis - not produced

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26
Q

plasmin function

A

cleaves serum fibrinogen

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27
Q

presentation of disorder of fibrinolysis?

A
  • increased PT/PTT (plasmin destroys coag factors)
  • increased bleedining time (blocks aggregation)
  • increased fibrinogen split products with NORMAL D-dimer
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28
Q

why is d-dimer normal in disorders of fibrinolysis

A

Fibrin thrombi are absent in the case of disorder of fibrinolysis therefore D-dimers are not formed

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29
Q

treatment for disorders of fibrinolysis?

A

aminocaproic acid — blocks activation of plasminogen

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30
Q

A deficiency of cystathione beta synthase results in high levels of…

A

homocysteine

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31
Q

estrogen induces increased production of…

A

coagulation factors

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32
Q

name the microcytic anemias

A
  • iron deficiency
  • anemia of chronic disease
  • sideroblastic anemia
  • thalasemmia
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33
Q

what is the cofactor needed for ALAS?

A

B6

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34
Q

How does lead poisoning cause sideroblastic anemia?

A

-lead inhibits ALAD and ferrochelatase - needed for heme synthesis

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35
Q

if you have alpha thalasemia with 3 genes deleted, what will your Beta chains for?

A

tetramers - HbH - will damage RBCs

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36
Q

if you have alpha thalasemia with 4 genes deleted, what will form?

A

Hbarts - tetramers of gamma chains

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37
Q

How does alpha thalesemia with 4 genes deleted present?

A

Death in utero via hydrops fetalis

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38
Q

if you have alpha thalasemia with two genes deleted how will you present?

A

mild anemia with increased RBC count

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39
Q

alpha thalassemia is usually due to a gene…

A

deletion

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40
Q

beta thalassemia is usualy due to a gene…

A

mutation (point mutation)

41
Q

what is the genotype of someone with beta thalasemia minor?

A

One normal beta globin gene, one mutated gene with diminished production
B/B+

42
Q

hemoglobin electrophoresis findings wiht beta thalasemmia minor?

A
  • Slightly decreased HbA

- Increased HbA2 and HbF

43
Q

what is the most severe form of beta thalesemia? and genotype?

A

Beta thalassemia major - Bo/Bo

Both mutations yeild absent production

44
Q

explain the pathophys of beta thalasemia major?

A

No production of Beta chains — alpha chains precipitate and damage RBC membranes causing ineffective erytrhopoiesis and extravascular hemolysis
-Massive erythroid hyperplasia

45
Q

presentation of beta thalasemia major?

A
  • crewcut appearance on xray
  • chipmunk facies
  • extramedullary hematopoiesis with hepatosplenomegaly
  • risk of aplastic crisis with parvovirus B19
46
Q

electrophoresis findings of someone with beta thalasemia major?

A
  • No HbA (or little)

- HbA2 and HbF

47
Q

target cells are characteristic of which disease?

A

beta thalasemmias and sickle cell

48
Q

inheritance of paroxysmal nocturnal hemoglobinuria?

A

NOT inherited - its AQUIRED

49
Q

what is defective in paroxysmal nocturnal hemoglobinuria?

A

GPI - the protein that anchors decay accelerating factor to the surface of RBCs, preventing C3 convertase activity

50
Q

decay accelerating factor =

A

CD55

51
Q

what is the main cause of death in patients with paroxysmal nocturnal hemoglobinuria?

A

thrombosis - destroyed platelets release cytoplasmic contents -> thrombosis

52
Q

how do blasts (immature cells) appear on blood smear?

A

Large immature cells often with punched out nuceloili

53
Q

what do lymphoblasts stain for?

A

Tdt - a DNA polymerase

54
Q

is Tdt postive in myeloid blasts or mature lymphocytes?

A

no - specific to lymphoblasts

55
Q

what do myeloblasts stain positive for?

A

MPO (in the cytoplasmi) - may crystalize and be seen as auer rods

56
Q

what translocation characterizes acute promyelocytic leukemia?

A

t(15:17) -translocation of retinoic acid receptors (RAR) on chromosome 17 to chromosome 15 ; blocks maturation and promyelocytes accumulate

57
Q

Abnormal promyelocytes increase your risk…

A

DIC, as they contain granules

58
Q

CLL is a proliferation of what?

A

Naive B cells

59
Q

what do the naive B cells of CLL express?

A

both CD5 and CD20

60
Q

What will be seen on blood smear of someone with CLL?

A

increased lymphocytes and smudge cells

61
Q

complications of CLL?

A
  • Hypogammaglobulinemia - may lead to infection
  • autoimmune hemolytic anemia
  • transformation to diffuse large B-cell lymphoma (richter transformation)
62
Q

what is hairy cell leukemia a proliferation of?

A

mature B cells

63
Q

how do the mature B cells in hairy cell leukemia appear?

A

they have hairy cytoplasmic processes

64
Q

what do the mature B cells in hairy cell leukemia stain for?

A

TRAP

65
Q

clinical features of hairy cell leukemia

A
  • splenomegaly - cells accumulate in red pulp

- dry tap on bone marrow due to fibrosis

66
Q

what is adult t-cell leukemia/lymphoma a proliferation of?

A

mature CD4 t cells

67
Q

adult t-cell leukemia/lymphoma is associated with which virus?

A

HTLV-1

68
Q

what is mycosis fungoides a proliferation of?

A

mature CD4 t cells

69
Q

mycosis fungoides clinical features

A

the CD4 t cells infiltrate the skin, leading to rashes, plaques, etc. Pautrier microabscesses.
-may spread to involve the blood = sezary syndrome

70
Q

where do B cell proliferate in the lymph node?

A

cortex

71
Q

where do t cells proliferate in th elymph node?

A

paracortex

72
Q

hyperplasia of the sinus histiocytes is seen…

A

in lymph nodes that are draining a tissue with cancer

73
Q

which lymphomas arise from small B cells?

A
  • follicular lymphoma
  • mantle cell lymphoma
  • marginal cell lymphoma
  • small lymphocytic lympoma (CLL that involes tissue)
74
Q

which lymphomas arise from intermediate B cells

A

burkitt lymphoma

75
Q

which lymphomas arise from large B cells

A

large B cell lympoma

76
Q

follicular lympoma is a neoplasitc proliferation of…

A

small b cells (CD20+)

77
Q

what translocation drives follicular lympoma?

A

t(14:18)

78
Q

what does the t(14:18) result in?

A

BCL2 on chromosome 18 translocates to the ig heavy chain locus on chromosome 14- > overexpression of Bcl2 which inhibits apoptosis

79
Q

which treatment is useful in follicular lymphoma?

A

rituximab

80
Q

what is mantle cell lymphoma a proliferation of?

A

small b cells (CD20+)

81
Q

what transcolation drives mantel cell lymphoma?

A

t(11:14)

82
Q

what does the t(11:14) result in?

A

cyclin D1 from chromosme 11 gets translocated to ig heavy chian on chromsome 14 -> overexpression of cyclin D1 which promotes G1/S transition

83
Q

what is marginal zone lymphoma a proliferation of?

A

small b cells (CD20+)

84
Q

what translocation drives mantle cell lymphoma?

A

t(11:18)

85
Q

what is MALToma?

A

a marginal zone lymphoma in mucosal sites

86
Q

what conditions is marginal zone lymphoma associated with?

A

Hashimoto thryoiditis, Sjogren syndrome, and H pylori

87
Q

which translocation drives burkitts lymphoma?

A

t(8:14)

88
Q

what does the t(8:14) result in?

A

c-myc translocation onto the Ig heavy chain locus on chromosome 14 -> overexpression of c-myc oncogene

89
Q

what is the most common type of hodgkins lymphoma?

A

nodular sclerosis

90
Q

how will a lymph node with the nodular sclerosis type of hodgkins lymphoma appear?

A

divided by bands of sclerosis, with reed sternberg cells in lake like spaces

91
Q

what cytokine do RS cells produce?

A

IL-5

92
Q

which cytokine may be increased in multiple myeloma?

A

IL6

93
Q

what does the M spike seen in multiple myeloma represent?

A

monoclonal IgG or igA produced by malignant plasma cells

94
Q

why does the rouleaux formation occur in multiple myeloma?

A

-the elavated serum protein (from the neoplastic plasma cells producing immunoglobulins) decrease the charge between RBCs

95
Q

which type of amyloid is found in multiple myeloma?

A

primary AL amyloidosis

96
Q

what is waldenstrom macroglobulinemia?

A

b-cell lymphoma with monoclonal igM production

97
Q

what do the langherhans cells of langerhans cell histiocytosis stain + for?

A

CD1a+ and S-100

98
Q

presentation of hand-schuller-christain disease?

A

scalp rash, lytic skull defects, diabetes insipidus, exopthalamus in a child

Test reviews DEDICATED (70 decks)

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