Hematology 2

Front 1

A peptidase enzyme that converts procollagen to collagen

Back 1

Ehlers-Danlos syndrome

Front 2

Connective tx elastic fibers in small arteries are calcified and structurally abnormal

Back 2

PseudoXanthoma Elasticum

Hint 2

Autosomal Recessive

Front 3

Required for the formation of the intact structure 9f the vascular basement membrane

Back 3

Vit. C Deficiency (scurvy)

Hint 3

Deficiency of vit. C ascorbic acid

Front 4

The aging process, brings about degeneration of collagen, elastin and subcutaneous fat

Back 4

Senile Purpura

Hint 4

Chronic disease of elderly

Front 5

Characterized by vascular malformation and surface skin lesions

Back 5

Hereditary hemorrhagic telangiectasia

Hint 5

Osler weber rendu disease

Front 6

Other name for Hereditary Hemorrhagic Telangiectasia

Back 6

Osler Weber Rendu Disease

Front 7

Disorders associated with tumors composed of vessels that commonly swell and bleed at the surface

Back 7

Congenital hemangiomata

Hint 7

Kasabach-Meritt Syndrome

Front 8

Large vessels may become atheroslerotic and capillary basement membrane may thicken (blocking the normal flow of blood)

Back 8

Diabetes Mellitus

Hint 8

Affected: capillaries of renal glomeruli & retina

Front 9

There is deposition of fibrillar protein, which causes various degrees of vessel obstruction

Back 9

Amyloidosis

Front 10

Common drugs that induced purpura:

Back 10

Quinine

Procaine penicillin

Aspirin

Sulfonamides

Sedatives

Coumarins

Hint 10

Drug-Induced Purpura

Front 11

Caused by certain foods, drugs, cold, insect bites and vaccination

Back 11

Allergic Purpura

Hint 11

Anaphylactoid Purpura

Front 12

Other name for Congenital Hemangiomata

Back 12

Kasabach-Meritt Syndrome

Front 13

Other name for Allergic Purpura

Back 13

Anaphylactoid Purpura

Front 14

Purpura with abdominal pain sec9ndary to GIT bleeding associated with hemathrosis

Back 14

Henoch-Schonlein Purpura

Front 15

Characterized by Hemorrhagic Manifestation following a streptococcal infection

Back 15

Purpura Fulminans

Front 16

Characterized by capillary damage following meningococcal septicemia

Back 16

Waterhouse-Friedrichsen Syndrome

Front 17

Rocky mountain spotted fever

Back 17

Ricketssial

Front 18

Other name for Bernard-Soulier Syndrome

Back 18

Giant platelet syndrome

Front 19

Platelets lack the membrane receptor Gp1b necessary for them to bind with vwf

Back 19

Bernard-soulier syndrome

Hint 19

Defect in platelet

Front 20

Laboratory findings for Bernard-Soulier Syndrome

Back 20

NORMAL

ADP

Collagen

Epinephrine

ABNORMAL

Ristocetin

Front 21

Deficiency or defect in plasma VIII:vwf

Back 21

Von Willebrand's disease

Front 22

Laboratory findings for Von Willebrand's disease

Back 22

NORMAL

ADP

Collagen

Epinephrine

ABNORMAL

Ristocetin

Front 23

Deect in Gp IIb/IIIa

Back 23

Glanzmann's Thrombasthemia

Front 24

Laboratory findings for Glanzmann's Thrombasthenia

Back 24

NORMAL

Ristocetin

ABNORMAL

ADP

Collagen

Epinephrine

Front 25

Plasma defect that will cause a platelet aggregation disorder similar to Glanzmann's Thrombastenia

Back 25

Afibrinogenemia

Front 26

Defect lysosomal function in a variety of cell types, ceroid-like deposition in the cells of RES and a profound platelet dense granule deficiency

Back 26

Hermansky -pudlak syndrome

Front 27

Characterized by partial oculocutaneous albinism, frequently pyogenic bacterial infections, giant lysosomal granules, platelet dense granule deficiency amd hemorrhage

Back 27

Chediak-Higashi Syndrome

Front 28

Platelets are structurally abnormal, lacking in storage pool nucleotides, the # of dense granule is decreased and platelets are small

Back 28

Wiskott-aldrich syndrome

Front 29

Characterized by the triad of severe eczema, recurrent infections, and life threatening thrombocytopenia

Back 29

Wiskott-aldrich syndrome

Front 30

Cogenital absence or extreme hypoplasia of radial bones, numerous cardiac and other skeletal abnormalities, and thrombocytopenia

Back 30

Thrombocytopenia with absent radii (TAR) syndrome

Front 31

A large platelet whose ray appearance on a Wright-stained blood film

Back 31

Gray platelet syndrome

Front 32

Characterized by the specific absence of morphologically recognizable alpha granules in platelets

Back 32

Gray platelet syndrome

Front 33

Both alpha and dense granules are deficient

Back 33

Alpha-delta storage pool deficiency

Front 34

Deficiency of multimerin and shows abnormal proteolysis and deficiency of alpha granules

Back 34

Quebec platelet disorder

Front 35

Due to decreased surface expression of phosphatidylserine (PS)

Back 35

Scott syndrome

Front 36

Platelets are always in an activated state and express phosphatidylserine on the outer leaflet of the membrane without prior activation

Back 36

Stormorken syndrome

Front 37

Platelet cout below the lower limit of the reference interval

Back 37

Thrombocytopenia

Front 38

Platelet ct above the upper limit of the reference interval

Back 38

Thrombocytosis

Front 39

Abnormal high platelet ct with dysfunctional platelets

Back 39

Thrombocythemia

Front 40

Mutations in the MYH9 gene that encodes for nonmuscle myosin heavy chain

Back 40

May-Hegglin Anomaly

Front 41

Large platelets, thrombocytopenia, granulocytic inclusions

Back 41

Sebastian syndrome

Front 42

Similar abnormalities w/ sebastian syndrome accompanied by deafness, ocular problems, & glomerular nephritis

Back 42

Epstein syndrome

Front 43

Cause by mutation in c-mIp gene resulting complete loss of thrombopoeitin receptor function

Back 43

Congenital Amegakaryocytic thrombocytopenia

Front 44

Infection with TORCH, HIV infection, and in Utero exposure to certain drugs

Back 44

Neonatal hypoplasia

Front 45

Chemotherapeutic agents suppress bone marrow megakaryocyte production

Back 45

Acquired (drug-induced) hypoplasia

Hint 45

Ingestion of ETHANOL

Front 46

Usual feature of megaloblastic anemias

Back 46

Ineffective thrombopoiesis

Front 47

Platelets may be destroyed as a result of their interaction with products of RBC breakdown

Back 47

Hemolytic disease of the newborn

Front 48

Characterized by the triad of MAHA, thrombocytopenia, and neurologic abnormalities

Back 48

Thrombotic thrombocytopenic purpura

Front 49

Lack of neurologic symptoms, presence of renal dysfunction absence of other organ involvement

Back 49

Hemolytic uremic syndrome

Front 50

Destructive thrombocytopenia is activation of the coagulation cascade, resulting in aconsumptive coagulopathy

Back 50

Disseminated intravascular coagulation

Front 51

Elevation in the platelet count secondary to inflammation, trauma, or other underlying and seemingly unrelated conditions

Back 51

Reactive thrombocytosis

Front 52

Other name for reactive thrombocytosis

Back 52

Secondary thrombocytosis

Front 53

Platelet counts between 450,000/mcl & 800,000/mcl with no change in platelet function

Back 53

Reactive thrombocytosis

Front 54

Markedly and persistent elevation of plt ct.

Back 54

Autonomous thrombocytosis

Front 55

Other name for autonomous thrombocytosis

Back 55

Primary thrombocytosis

Front 56

Platelet counts exceeding 1M/mcl And uncontrolled proliferation of marrow megakaryocytes

Back 56

Essential thrombocythemia