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Histopathology Lec&Lab > MIDTERMS HEMA LAB > Flashcards

MIDTERMS HEMA LAB Flashcards

1
Q 
Rendu-Osler Weber syndrome
A. Hereditary Hemorrhagic Telangiectasia
B. Ataxia-telangiectasia
C. Pseudoxanthoma elasticum (PXE)
D.  Osteogenesis Imperfecta
A

A

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2
Q 
Vascular defect: thin-walled blood vessels with a discontinuous endothelium, inadequate smooth muscle and inadequate or missing elastin in the surrounding stroma
A. Hereditary Hemorrhagic Telangiectasia
B. Ataxia-telangiectasia
C. Pseudoxanthoma elasticum (PXE)
D.  Osteogenesis Imperfecta
A

A

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3
Q 
Diagnosis: based on the characteristic skin or mucous membrane lesions, a history of repeated hemorrhage, and a family history of a similar disorder
A. Pseudoxanthoma elasticum (PXE)
B. Ataxia-telangiectasia
C. Hereditary Hemorrhagic Telangiectasia
D.  Osteogenesis Imperfecta
A

C

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4
Q 
common in older men and women
A. Pseudoxanthoma elasticum (PXE)
B. Ataxia-telangiectasia
C. Hereditary Hemorrhagic Telangiectasia
D.  Cherry-red Hemangiomas
A

D

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5
Q 
Most common type of acquired vascular proliferation of the skin
A. Pseudoxanthoma elasticum (PXE)
B. Ataxia-telangiectasia
C. Hereditary Hemorrhagic Telangiectasia
D.  Cherry-red Hemangiomas
A

D

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6
Q 
They present as a dome-shaped, bright, ruby-colored papule with a pale halo
A. Pseudoxanthoma elasticum (PXE)
B. Ataxia-telangiectasia
C. Hereditary Hemorrhagic Telangiectasia
D.  Cherry-red Hemangiomas
A

D

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7
Q 
Louis-Bar syndrome
A. Pseudoxanthoma elasticum (PXE)
B. Ataxia-telangiectasia
C. Hereditary Hemorrhagic Telangiectasia
D.  Cherry-red Hemangiomas
A

B

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8
Q

Rare autosomal recessive condition characterized by:
★ Cutaneous telangiectasias
★ Cerebellar atrophy with progressive ataxia
★ Higher incidence of malignancy, immune deficiency, radio sensitivity, recurrent sinopulmonary infections
★ Elevated levels of alpha-fetoprotein (AFP) in serum
A. Pseudoxanthoma elasticum (PXE)
B. Ataxia-telangiectasia
C. Hereditary Hemorrhagic Telangiectasia
D. Cherry-red Hemangiomas

A

B

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9
Q 
Kasabach-Merritt syndrome
A. Pseudoxanthoma elasticum (PXE)
B. Hemangioma-thrombocytopenia syndrome
C. Hereditary Hemorrhagic Telangiectasia
D.  Cherry-red Hemangiomas
A

B

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10
Q 
Hereditary basis: NOT established, but the condition is present at birth
A. Pseudoxanthoma elasticum (PXE)
B. Hemangioma-thrombocytopenia syndrome
C. Hereditary Hemorrhagic Telangiectasia
D.  Cherry-red Hemangiomas
A

B

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11
Q

➔ Association of a giant cavernous hemangioma (vascular tumor), thrombocytopenia and a bleeding diathesis
➔ Complications: acute or chronic disseminated intravascular coagulation, sequestration of platelets in the hemangiomas and resultant microangiopathic hemolytic anemia
A. Pseudoxanthoma elasticum (PXE)
B. Hemangioma-thrombocytopenia syndrome
C. Hereditary Hemorrhagic Telangiectasia
D. Cherry-red Hemangiomas

A

B

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12
Q 
autosomal dominant, recessive or X-linked trait
A. Pseudoxanthoma elasticum (PXE)
B. Hemangioma-thrombocytopenia syndrome
C. Ehlers-Danlos Syndrome
D.  Cherry-red Hemangiomas
A

C

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13
Q

➔ Manifestations: hyper-extensible skin, hypermobile joints, joint laxity, fragile tissues, and a bleeding tendency, primarily subcutaneous hematoma formation
➔ Defects: collagen production, structure or crosslinking with resulting inadequacy of the connective tissues
A. Pseudoxanthoma elasticum (PXE)
B. Hemangioma-thrombocytopenia syndrome
C. Ehlers-Danlos Syndrome
D. Cherry-red Hemangiomas

A

C

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14
Q 
Progressive disorder that is characterized by the accumulation of deposits of calcium and other minerals (mineralization) in elastic fibers
A. Pseudoxanthoma elasticum (PXE)
B. Hemangioma-thrombocytopenia syndrome
C. Ehlers-Danlos Syndrome
D.  Cherry-red Hemangiomas
A

A

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15
Q 
An inherited diseases that affects the body’s connective tissue which gives strength, support and elasticity to tendons, cartilage, heart valves, blood vessels and other vital parts of your body
A. Pseudoxanthoma elasticum (PXE)
B. Marfan Syndrome
C. Ehlers-Danlos Syndrome
D.  Cherry-red Hemangiomas
A

B

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16
Q 
➔ A genetic disorder that causes a person’s bones to break easily, often from little or no apparent trauma
➔ OI is called “brittle bone disease
A. Osteogenesis Imperfecta
B. Marfan Syndrome
C. Ehlers-Danlos Syndrome
D.  Cherry-red Hemangiomas
A

A

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17
Q 
An acute IgA-mediated disorder with widespread generalized vasculitis involving the skin, joints, kidneys, gastrointestinal tract, and less commonly the lungs
A. Henoch-Schonlein purpura
B. Marfan Syndrome
C. Ehlers-Danlos Syndrome
D.  Amyloidosis
A

A

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18
Q 
The purpuric skin lesions are frequently confused with the hemorrhagic rash of immune thrombocytopenic purpura
A. Henoch-Schonlein purpura
B. Senile Purpura
C. Ehlers-Danlos Syndrome
D.  Amyloidosis
A

A

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19
Q 
Myeloma proteins – inhibits platelet function
A. Henoch-Schonlein purpura
B. Marfan Syndrome
C. Paraproteinemia
D.  Amyloidosis
A

C

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20
Q 
Increased concentration of Paraproteins – cause severe hemorrhagic manifestations as a result of a combination of hyperviscosity and platelet dysfunction
A. Henoch-Schonlein purpura
B. Marfan Syndrome
C. Paraproteinemia
D.  Amyloidosis
A

C

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21
Q 
About ⅓ of patients with IgA Myeloma and Waldenstrom macroglobulinemia and 5% of patient with IgG (IgG3) Myeloma – exhibits platelet function abnormalities
A. Henoch-Schonlein purpura
B. Marfan Syndrome
C. Paraproteinemia
D.  Amyloidosis
A

C

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22
Q 
Deposition of abnormal quantities of amyloid protein in tissues, may be primary or secondary and localized or systemic
A. Henoch-Schonlein purpura
B. Senile Purpura
C. Ehlers-Danlos Syndrome
D.  Amyloidosis
A

D

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23
Q 
Clinical presentation: purpura, hemorrhage, thrombosis, abnormal platelet function
A. Henoch-Schonlein purpura
B. Senile Purpura
C. Ehlers-Danlos Syndrome
D.  Amyloidosis
A

D

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24
Q

➔ More commonly in elderly men than women
➔ Due to a lack of collagen support for small blood vessels and loss of subcutaneous fat and elastic fibers
A. Henoch-Schonlein purpura
B. Senile Purpura
C. Ehlers-Danlos Syndrome
D. Amyloidosis

A

B

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25
Q 
Associated with drug-induced vasculitis occurs in the presence of functionally adequate platelets
A. Drug-Induced vascular purpuras
B. Senile Purpura
C. Ehlers-Danlos Syndrome
D.  Amyloidosis
A

A

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26
Q

Drugs are known to cause vascular purpura:

A 
aspirin
warfarin,
barbiturates
diuretics
digoxin
methyldopa
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27
Q 
Sulfonamides and iodides have been implicated most frequently
A. Drug-Induced vascular purpuras
B. Senile Purpura
C. Ehlers-Danlos Syndrome
D.  Amyloidosis
A

A

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28
Q 
Patients may present with isolated thrombocytopenia or in conjunction with characteristic syndromes
A. Congenital Thrombocytopenias
B. Senile Purpura
C. Autosomal Recessive Thrombocytopenias
D.  Amyloidosis
A

A

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29
Q

➔ Patients may have platelet functional defects
➔ Features: increase in platelet size, cytoplasmic inclusions in leukocytes (Dohle bodies) and premature release of platelets
A. Congenital Thrombocytopenias
B. Senile Purpura
C. Autosomal Recessive Thrombocytopenias
D. Amyloidosis

A

A

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30
Q 
Diverse group of autosomal dominant syndromes referred to as: MYH9-related macrothrombocytopenia which includes
A. Congenital Thrombocytopenias
B. Autosomal Dominant Thrombocytopenias
C. Autosomal Recessive Thrombocytopenias
D.  Amyloidosis
A

B

31
Q

MYH9-related macrothrombocytopenia includes

A
  1. May-Hegglin anomaly
  2. Fetchner syndrome
  3. Epstein syndrome
  4. Sebastian syndrome
32
Q

Types under Autosomal Recessive Thrombocytopenias

A

CAMT
Gray Platelet syndrome
TAR

33
Q

➔ Autosomal recessive disorder associated with mutations in the thrombopoietin receptor MPL
➔ Severe thrombocytopenia
➔ Absence of megakaryocytes in the bone marrow
A. Congenital Thrombocytopenias
B. Autosomal Dominant Thrombocytopenias
C. Autosomal Recessive Thrombocytopenias
D. Amyloidosis

A

C

34
Q 
Mutations in NBEAL2 gene – a BEACH protein involved in vesicular trafficking
A. Congenital Thrombocytopenias
B. Autosomal Dominant Thrombocytopenias
C. Autosomal Recessive Thrombocytopenias
D.  Gray Platelet syndrome
A

D

35
Q 
Associated with skeletal abnormalities
A. Congenital Thrombocytopenias
B.  Thrombocytopenia with Absent Radii (TAR)
C. Autosomal Recessive Thrombocytopenias
D.  Gray Platelet syndrome
A

B

36
Q

➔ X-linked thrombocytopenia have mutations in the WAS gene
➔ Small platelets, thrombocytopenia
A. Wiskott-Aldrich Syndrome (WAS)
B. Thrombocytopenia with Absent Radii (TAR)
C. Autosomal Recessive Thrombocytopenias
D. Gray Platelet syndrome

A

A

37
Q
  • Heterogeneous group of disorders characterized byautoimmune-mediated platelet destruction
  • Associated with impaired platelet production

A. Wiskott-Aldrich Syndrome (WAS)
B. Immune thrombocytopenia
C. Autosomal Recessive Thrombocytopenias
D. Gray Platelet syndrome

A

B

38
Q 
Diagnosis of exclusion, made in the absence of other causes or disorders associated with thrombocytopenia
A. Wiskott-Aldrich Syndrome (WAS)
B. Primary ITP
C. Secondary ITP
D.  Gray Platelet syndrome
A

B

39
Q 
Consists of all forms of immune-mediated thrombocytopenia except primary ITP
A. Wiskott-Aldrich Syndrome (WAS)
B. Primary ITP
C. Secondary ITP
D.  Gray Platelet syndrome
A

C

40
Q

Rare type of thrombocytosis

A

congenital

41
Q 
Results from gain-of-function mutations in either thrombopoietin or its receptor (MPL)
A. Wiskott-Aldrich Syndrome (WAS)
B. Primary ITP
C. Congenital Thrombocytosis
D.  Gray Platelet syndrome
A

C

42
Q

Common type of thrombocytosis

A

Acquired

43
Q

2 type of acquired thrombocytosis

A

Primary and essential

44
Q 
Related to autonomous clonal bone marrow disorder (Myeloproliferative neoplasms MPN)
A. Wiskott-Aldrich Syndrome (WAS)
B. Primary ITP
C. Congenital Thrombocytosis
D. Primary Thrombocytosis
A

D

45
Q 
Secondary to conditions such as infections, chronic inflammation, malignancy, hemolysis, iron-deficiency anemia or splenectomy
A. Wiskott-Aldrich Syndrome (WAS)
B. Essential Thrombocytosis
C. Congenital Thrombocytosis
D. Primary Thrombocytosis
A

B

46
Q

A reactive acquired thrombocytosis

A

Essential thrombocytosis

47
Q

Rare disorder of platelet adhesion that usually manifests in infancy or childhood with hemorrhage characteristics of defective platelet function: ecchymoses, epistaxis and gingival bleeding
A. Glanzmann Thrombasthenia
B. Essential Thrombocytosis
C. Bernard-Soulier (giant platelet) syndrome
D. Primary Thrombocytosis

A

C

48
Q

MOI of BSS

A

autosomal recessive disorder

49
Q

Inability to bind to VWF accounts for the inability of platelets to adhere to exposed sub-endothelium and the resultant bleeding characteristic of the disorder
A. Glanzmann Thrombasthenia
B. Essential Thrombocytosis
C. Bernard-Soulier (giant platelet) syndrome
D. Primary Thrombocytosis

A

C

50
Q

Defect in adhesion shows the importance of initial platelet attachment for primary hemostasis
A. Glanzmann Thrombasthenia
B. Essential Thrombocytosis
C. Bernard-Soulier (giant platelet) syndrome
D. Primary Thrombocytosis

A

C

51
Q

Laboratory feature: Platelet aggregation (N) in response to ADP, epinephrine, collagen, arachidonic acid
A. Glanzmann Thrombasthenia
B. Essential Thrombocytosis
C. Bernard-Soulier (giant platelet) syndrome
D. Primary Thrombocytosis

A

C

52
Q

Lack of response to: ristocetin (due to lack of GP1b/IX/V complexes
A. Glanzmann Thrombasthenia
B. Essential Thrombocytosis
C. Bernard-Soulier (giant platelet) syndrome
D. Primary Thrombocytosis

A

C

53
Q

BSS has diminished response to

A

thrombin

54
Q

Abnormal in vitro clot retraction and a normal platelet count
A. Glanzmann Thrombasthenia
B. Essential Thrombocytosis
C. Bernard-Soulier (giant platelet) syndrome
D. Primary Thrombocytosis

A

A

55
Q 
Deficient/Abnormal in GPIIb/IIIa
A. Glanzmann Thrombasthenia
B. Essential Thrombocytosis
C. Bernard-Soulier (giant platelet) syndrome
D. Primary Thrombocytosis
A

A

56
Q

Laboratory feature: Platelet Count (N), morphology (N)
A. Glanzmann Thrombasthenia
B. Essential Thrombocytosis
C. Bernard-Soulier (giant platelet) syndrome
D. Primary Thrombocytosis

A

A

57
Q

Lack of platelet aggregation (in response to ADP, collagen, thrombin and epinephrine)
A. Glanzmann Thrombasthenia
B. Essential Thrombocytosis
C. Bernard-Soulier (giant platelet) syndrome
D. Primary Thrombocytosis

A

A

58
Q

Stimulation of strong antagonist (THROMBIN): will induce the release/secretion reaction, even in the absence of aggregation
A. Glanzmann Thrombasthenia
B. Essential Thrombocytosis
C. Bernard-Soulier (giant platelet) syndrome
D. Primary Thrombocytosis

A

A

59
Q

Normal: Ristocetin-induced binding of VWF to platelets and the resulting platelet agglutination
A. Glanzmann Thrombasthenia
B. Essential Thrombocytosis
C. Bernard-Soulier (giant platelet) syndrome
D. Primary Thrombocytosis

A

A

60
Q 
Specific absence of morphologically recognizable a-granules in platelets
A. Gray platelet syndrome
B. Essential Thrombocytosis
C. Hermansky-Pudlak syndrome
D. Chediak-Higashi syndrome
A

A

61
Q

Lifelong mild bleeding tendencies, moderate thrombocytopenia, fibrosis of the marrow and large platelets whose gray appearance (wright stained blood film is the source of the name of this disorder)

A. Gray platelet syndrome
B. Essential Thrombocytosis
C. Hermansky-Pudlak syndrome
D. Chediak-Higashi syndrome

A

A

62
Q 
Gene mutation: region 3p21.31 involving the gene NBEAL2 have been identified (crucial for the development of a-granules)
A. Gray platelet syndrome
B. Essential Thrombocytosis
C. Hermansky-Pudlak syndrome
D. Chediak-Higashi syndrome
A

A

63
Q

Tyrosinasepositive oculocutaneous albinism, defective lysosomal function in a variety of cell types, ceroid-like deposition in the cells of the reticuloendothelial system and a profound platelet dense granule deficiency

A. Gray platelet syndrome
B. Essential Thrombocytosis
C. Hermansky-Pudlak syndrome
D. Chediak-Higashi syndrome

A

C

64
Q 
Hermansky-Pudlak syndrome gene mutation
A. Chromosome 1
B. Chromosome 9
C. Chromosome 19
D. Chromosome 24
A

C

65
Q 
Partial oculocutaneous albinism, frequent pyogenic bacterial infections, giant lysosomal granules in cells of hematologic and non-hematologic origin, platelet dense granule deficiency and hemorrhage
A. Gray platelet syndrome
B. Essential Thrombocytosis
C. Hermansky-Pudlak syndrome
D. Chediak-Higashi syndrome
A

D

66
Q 
Accompanied by severe immunologic defects and progressive neurologic dysfunction in patients who survive to adulthood
A. Gray platelet syndrome
B. Essential Thrombocytosis
C. Hermansky-Pudlak syndrome
D. Chediak-Higashi syndrome
A

D

67
Q 
Chediak-Higashi syndrome gene mutation
A. Chromosome 1
B. Chromosome 9
C. Chromosome 19
D. Chromosome 24
A

A

68
Q 
rare X-linked disease
A. Gray platelet syndrome
B. Essential Thrombocytosis
C. Wiskott-Aldrich syndrome
D. Chediak-Higashi syndrome
A

C

69
Q 
Gene mutation: WAS gene in the short arm of X chromosome Xp11.23
A. Gray platelet syndrome
B. Essential Thrombocytosis
C. Wiskott-Aldrich syndrome
D. Chediak-Higashi syndrome
A

C

70
Q 
Classic form: eczema-thrombocytopenia immunodeficiency syndrome- susceptibility to infections associated with immune dysfunction with recurrent bacterial, viral and fungal infections, microthrombocytopenia and severe eczema
A. Gray platelet syndrome
B. Essential Thrombocytosis
C. Wiskott-Aldrich syndrome
D. Chediak-Higashi syndrome
A

C

71
Q 
Has features of structurally abnormal platelets, decreased dense granules, small platelets (microthrombocytes)- feature of diagnostic importance, low aggregation: ADP, Collagen, epinephrine and N: thrombin 
A. Gray platelet syndrome
B. Essential Thrombocytosis
C. Wiskott-Aldrich syndrome
D. Chediak-Higashi syndrome
A

C

72
Q

All of the following are rare autosomal recessive disorder except:
A. Gray platelet syndrome
B. Chediak-Higashi syndrome
C. Hermansky-Pudlak syndrome
D. Thrombocytopenia Absent radius (TAR) syndrome

A

C

73
Q

Congenital absence of the radial bones (the most pronounced skeletal abnormality), numerous cardiac and other skeletal abnormalities, and thrombocytopenia (90% of cases)
A. Gray platelet syndrome
B. Chediak-Higashi syndrome
C. Hermansky-Pudlak syndrome
D. Thrombocytopenia Absent radius (TAR) syndrome

A

D

74
Q

Platelets have structural defects in dense granules, with corresponding abnormal aggregation responses

A. Gray platelet syndrome
B. Chediak-Higashi syndrome
C. Thrombocytopenia Absent radius (TAR) syndrome
D. Hermansky-Pudlak syndrome

A

C

Histopathology Lec&Lab (7 decks)

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