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HEMATOLOGY 2 > QUANTITATIVE PLATELENT DISORDERS > Flashcards

QUANTITATIVE PLATELENT DISORDERS Flashcards

1
Q

quantitative plt disorders has 2 categories/distinction, what are they

A

thrombocytopenia
thrombocytosis

since quantitative means NUMBER, so it will only talks about the decrease and increase number of platelets

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2
Q

Pathophysiologic Process that result in
Thrombocytopenia

A

decrease plt production
increase plt destruction
abnormal plt distribution

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3
Q

2 categories in abnormal platelet production

A

megakaryocyte hypoplasia
ineffective thrombopoiesis

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4
Q

MYH9 mode of inheritance

A

Inherited / Congenital Thrombocytopenia: Autosomal dominant thrombocytopenia

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5
Q

MYH9 related to thrombocytopenia syndromes is caused by the mutations in the ___

A

MYH9 gene

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6
Q

MYH9- related Thrombocytopenia
Syndromes examples

A

may-hegglin anomaly
sebastian syndrome
epstein syndrome
Fechtner syndrome

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7
Q

MYH9- related Thrombocytopenia
Syndromes

affected patients have a triad of

A

thrombocytopenia
macrothrombocytes
dohle body-like inclusions in the leukocytes except epstein syndrome

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8
Q

type of inclusion found in MYH9 related thrombocytopenia syndrome

A

dohle body like inclusions

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9
Q

among the conditions associated with MYH9 related thrombocytopenia syndrome, which one has no dohle body-like inclusions

A

Epstein syndrome

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10
Q

describe the thrombocytopenia of MYH9-related thrombocytopenia syndrome

A

mild to moderate thrombocytopenia

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11
Q

MYH9 related thrombocytopenia syndrome and its associated conditions will show _______ in peripheral blood smear

A

enlarge platelets with frequent giant platelets

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12
Q

MYH9- related Thrombocytopenia
Syndromes

example of conditions where we can observe macrothrombocytopenia

A

may hegglin
sebastian
fechtner
epstein

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13
Q

MYH9- related Thrombocytopenia
Syndromes

example of conditions where we can observe dohle-like bodies/ dohle body-like inclusions

A

may hegglin
fechtner
sebastian

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14
Q

MYH9- related Thrombocytopenia
Syndromes

example of conditions that we can observe in nephritis

A

fechtner
epstein

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15
Q

MYH9- related Thrombocytopenia
Syndromes

example of conditions that we can observe in deafness

A

fechtner
Epstein

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16
Q

MYH9- related Thrombocytopenia
Syndromes

example of conditions that we can observe in cataracts

A

fechtner

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17
Q

Thrombocytopenia with Absent Radius (TAR)

what is the mode of inheritance

A

autosomal recessive thrombocytopenia

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18
Q

Thrombocytopenia with Absent Radius (TAR) is associated with what mutation

A

RBM8A - CH1

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19
Q

Thrombocytopenia with Absent Radius (TAR) is a rare disease first identified in what year

A

1959

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20
Q

Thrombocytopenia with Absent Radius (TAR)

plt count is approximately

A

10-30 X 10^3/ul

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21
Q

Thrombocytopenia with Absent Radius (TAR)

describe the ff:

plt count:
serum tpo:
marrow cellularity:

A

plt count: approx 10-30 X 10^3/ul
serum tpo: normal
marrow cellularity: normal or increased

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22
Q

describe the megakaryocytes of Thrombocytopenia with Absent Radius (TAR)

A

low, absent or appears immature

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23
Q

Thrombocytopenia with Absent Radius (TAR) is can be managed by ___

A

blood transfusion

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24
Q

transcribe CAMT

A

Congenital Amegakaryocytic Thrombocytopenia
(CAMT)

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25
Q

Congenital Amegakaryocytic Thrombocytopenia
(CAMT)

what is the mode of inheritance?

A

autosomal recessive thrombocytopenia

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26
Q

Congenital Amegakaryocytic Thrombocytopenia
(CAMT)

mutation in ____ which will cause the loss of TPO receptor

A

MPL gene -CH1

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27
Q

Congenital Amegakaryocytic Thrombocytopenia
(CAMT)

has the mutation in MPL gene - ch 1 which will cause the loss of _____

A

TPO receptor

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27
Q

Congenital Amegakaryocytic Thrombocytopenia
(CAMT)

has the presence of ___
and absence of ____

A

presence of severe thrombocytopenia
absence of megakaryocytes in the bone marrow

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28
Q

Congenital Amegakaryocytic Thrombocytopenia
(CAMT)

a patient having this condition has a family history of _____ CAMT, and both parents ___ plt count and function

A

negative in CAMT
both parents have normal plt count and function

remember that this one is autosomal recessive thrombocytopenia.

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29
Q

describe the serum TPO of patient with CAMT

A

elevated thrombopoietin

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30
Q

a congenital thrombocytopenia which the patient will develop progressive marrow aplasia

A

Congenital Amegakaryocytic thrombocytopenia
(CAMT)

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31
Q

2 groups of Congenital Amegakaryocytic Thrombocytopenia
(CAMT)

A

GROUP CAMT I
GROUP CAMT II

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32
Q

this group of camt has more severe type of thrombocytopenia with constantly low platelet count and an early onset of pancytopenia

A

group camt 1

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33
Q

this group of CAMT has transient increase in platelet counts during the first year of life and a later or no development of pancytopenia

A

group CAMT II

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34
Q

Congenital Amegakaryocytic Thrombocytopenia
(CAMT)

mutation in ____ which will cause the incomplete megakaryocyte differentiation

A

ANKRD26 - CH 10

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35
Q

plt countt of Congenital Amegakaryocytic Thrombocytopenia
(CAMT)

A

20,000 to 100,000/ul

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36
Q

Congenital Amegakaryocytic Thrombocytopenia
(CAMT)

is characterized by ___ thrombocytopenia by a normal plt survival and normal number of bone megakaryocytes

A

mild

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37
Q

Congenital Amegakaryocytic Thrombocytopenia
(CAMT)

pbs will show ____

A

plt macrocytosis

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38
Q

Congenital Amegakaryocytic Thrombocytopenia
(CAMT)

describe the ff:

plt aggregation:
plt membrane glycoproteins:

A

plt aggregation: abnormal
plt membrane glycoproteins: normal

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39
Q

mode of inheritance of wiskott aldrich syndrom

A

x linked thrombocytopenia

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40
Q

Wiskott-Aldrich Syndrome is originally described at what year

A

1937

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41
Q

is the most consistent
feature of WASP-associated disease

A

microthrombocytopenia

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42
Q

Neonatal Thrombocytopenia

plt count

A

less than 150, 000/ul

43
Q

cause of Neonatal Thrombocytopenia

A

infection with toxoplasma, rubella, cytomegalovirus, herpes

44
Q

neonatal thrombocytopenia

Thrombocytopenia may be severe plt count of

A

70,000 /ul

45
Q

most common infectious agent of neonatal thrombocytopenia

A

cytomegalovirus

46
Q

Idiopathic Thrombocytopenic Purpura is also known as

A

immune thrombocytopenic purpura

47
Q

one of the most common disorders causing severe isolated thrombocytopenia

A

idiopathic thrombocytopenia purpura

48
Q

idiopathic thrombocytopenic purpura is caused by

A

autoantibody to the patient’s platelet

49
Q

Idiopathic Thrombocytopenic Purpura

no specific test to confirm the presence of ITP is rather the diagnosis of __

A

exclusion

50
Q

Idiopathic Thrombocytopenic Purpura

can it be present in both children and adults?>

A

yes

51
Q

Idiopathic Thrombocytopenic Purpura

present an initial plt count of less than __

A

20 X 10^9/L

52
Q

Idiopathic Thrombocytopenic Purpura

progress of the disease

A

self limiting, spontaneous remissions with or without therapy in majority of the patient

53
Q

Idiopathic Thrombocytopenic Purpura

what is used to decrease the period of thrombocytopenia

A

IgG and corticosteroids

54
Q

age group of chronic Idiopathic Thrombocytopenic Purpura

A

20-50 y/o

55
Q

Idiopathic Thrombocytopenic Purpura

chronic disease process with a greater predilection for what gender

A

women

56
Q

Idiopathic Thrombocytopenic Purpura

patient with mucosal bleeding typical of a ____

A

primary epistaxis
easy bruisability
petechiae

57
Q

treatment of choice for Idiopathic Thrombocytopenic Purpura

A

IVIG

58
Q

Idiopathic Thrombocytopenic Purpura

bone marrow is characterized by ___

A

increased or normal number of megakaryocytes

59
Q

Idiopathic Thrombocytopenic Purpura

measurement of _____ specific for platelet surface GP IIb/IIIa and Ib/IX may provide greater specificity but still are not diagnostic

A

antibodies (IgG )

60
Q

conventional therapies of Idiopathic Thrombocytopenic Purpura

A

splenectomy and corticosteroids

61
Q

age group of acute Idiopathic Thrombocytopenic Purpura

A

2-6 yr/o

62
Q

frequent causes of drug induced thrombocytopenia

A

quinine

63
Q

quinine has been recognized as one of the most frequent causes of drug induced thrombocytopenia where it act as a ___

A

hapten

64
Q

drug most frequently cited for drug induced immune thromboyctopenia

A

quinine, quinidine, salicylates, thiazides, sulfa drugs

65
Q

drug induced immune thrombocytopenia occures more frequently to what age group

A

elderly due to increased usage of medication

66
Q

purpura in drug induced immune thrombocytopenia will occur approx how many days of initial use of drug

A

7 days - but may occur within 3-5 days owing to anamnestic response

67
Q

Neonatal alloimmune is also known as

A

isoimmune neonatal thrombocytopenia

68
Q

it results from immunization of the mother by fetal plt antigen and placental transfer of maternal antibody

A

Neonatal alloimmune /
Isoimmune Neonatal Thrombocytopenia

69
Q

most often caused by maternal alloantiboes to the P1a1 antigen

A

Neonatal alloimmune /
Isoimmune Neonatal Thrombocytopenia

70
Q

Neonatal alloimmune /
Isoimmune Neonatal Thrombocytopenia

uncommon disorder, generally affecting the ____ child

A

first born

71
Q

Infants who develop this disorder appear normal at
birth but within a few hours develop scattered
petechiae and purpuric hemorrhages, with platelet
counts below 30 X 109
/L

A

Isoimmune Neonatal Thrombocytopenia

72
Q

results from passive transplacental transfer of antibodies from mother with ITP or SLE

A

neonatal autoimmune thrombocytopenia

73
Q

it can be used to predict the neonatal plt count in most cases, but the clinical manifestations are less sever than in NAITP

A

maternal disease severity and
plt count during pregnancy

74
Q

Neonatal Thrombocytopenia persist for weeks- months

A

1-2

75
Q

Neonatal Autoimmune
Thrombocytopenia

give the tx

A

requires no treatment

76
Q

it is developed after 1 week of transfusion of plt containing products

A

post transfusion purpura

77
Q

manifested by RAPID ONSET of severe thrombocytopenia - bleeding

A

post transfusion purpura

78
Q

post transfusion purpura

the most effective way in resolving bleeding

A

plasmapheresis

79
Q

post transfusion purpura

tx of choice

A

IVIG

80
Q

examples of quantitative plt disorder secondary (non immune )

A

thrombocytopenia in pregnancy
HIV infection
HDN
TTP
DIC
Drug induced

81
Q

defined by hypertension and proteinuria

A

pre-eclampsia

82
Q

usually becomes evident during second trimester and is a major contributor to maternal and fetal morbidity and mortality

A

preeclampsia

83
Q

defined by the occurrence of acute neurologic abnormalities in a preeclamptic woman during perpartum period

A

eclampsia

84
Q

Low level of ADAMTS13 is detected

A

thrombocytopenia during pregnancy and preeclampsia

85
Q

HELLPS syndrome is characterized by

A

hemolysis
elevated Liver enzymes
low Platelet count

86
Q

HELLP syndrome is hard to differentiate from

A

TTP - Thrombotic thrombocytopenic purpura
DIC - disseminated intravascular coagulation
HUS - hemolytic uremic syndrome

87
Q

gestational thrombocytopenia is commonly developed in the ___ trimester of pregnancy and does not cause bleeding in the mother or infant

A

3rd

88
Q

gestational thrombocytopenia

the plt count will go back to normal during ___

A

after the delivery

89
Q

appears to be correlated between CD4+ T Cell depletion, viral load in plasma and the occurrence of thrombocytopenia

A

HIV related thrombocytopenia

90
Q

viral infection of hematopoietic cells, altered marrow microenvironment of dysfunction of the RES contribute to ineffective thrombopoiesis in ___

A

HIV related thrombocytopenia

91
Q
  • Development of marrow fibrosis and marrow
    involvement by AIDS-related lymphoma may also
    lead to __.
A

thrombocytopenia

92
Q

effective course of action of HIV related thrombocytopenia

A

antiretroviral therapy

93
Q

in HIV related thrombocytopenia, aside from retroviral therapy, it is found to be an effective treatment

A

Iv IgG and anti-D globulin

94
Q

HIV related thrombocytopenia, ____ may be effective but have the potential to increase the risk of infection in immunocompromised individuals

A

corticosteroid

95
Q

HIV related thrombocytopenia therapy and treatment

A

antiretroviral therapy
intravenous IgG and anti-D globulin
corticosteroids
splenectomy

96
Q

thrombotic thrombocytopenic purpura

first described as a pentad signs and symptoms that includes:

A

thrombocytopenia
microangiopathic hemolytic anemia
fever
neurologic abnormalities
renal dysfunction

97
Q

are the characteristic pathologic feature and are found in multiple organs of patient with thrombotic thrombocytopenic purpura

A

hyaline microthrombi

98
Q

coagulation screening test and D-dimer assay in TTP and compare it to DIC

A

coagulation screening and D dimer assay are normal in TTP

in DIC - both are abnormal

99
Q
  • Two types of TTP
A

acquired and hereditary

100
Q

acquired thrombotic thrombocytopenic purpura, the thrombi is found mostly in what organs

A

heart, pancreas, spleen, kidney, adrenal gland, and brain and are mainly compose of plt and vWF

101
Q

Acquired Thrombotic Thrombocytopenic Purpura is associated with ___ deficiency

A

ADAMTS13

102
Q

a disintegrin-like and metalloprotease with thrombospondin motifs

A

ADAMTS13

103
Q

ADAMTS13 is also found deficient or decrease in what conditions

A

sepsis, DIC, and liver disease

104
Q

acquired TTP

thrombocytopenia and hemolysis, with blood smear showing ___

A

polychromasia,
basophilic stippling
nucleated cells
schistocytes

105
Q

describe the retics in TTP

A

increased

106
Q
A

HEMATOLOGY 2 (18 decks)

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