Quantitative Platelet Disorders (M)

Question 1

What are quantitative PLT disorders?

Answer 1

These are disorders affecting PLT ct resulting to thrombocytopenia or thrombocytosis

Question 2

Answer the ff questions:

Given disease: Congenital Amegakaryocytic Thrombocytopenia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 2

1) None
2) Autosomal recessive
3) a. Decreased production of PLTs
b. Loss of TPO receptor function
4) Mutations in the MPL gene of chromosome 1
5) a. Petechiae
b. Evidence of bleeding
c. Frequent physical anomalies
d. Aplastic anemia during 1st yr of life
e. Myelodysplasia and leukemia during childhood
6) a. Plasma TPO: increased
b. Megakaryocyte progenitors: decreased
7) Allogenic stem cell transplantation

Question 3

Answer the ff questions:

Given disease: Autosomal Dominant Thrombocytopenia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 3

1) None
2) Autosomal dominant
3) a. Decreased production of PLTs
b. INC megakaryocyte differentiation
4) Mutations in the ANKRD26 gene of chromosome 10
5) Absent or mild bleeding
6) a. PLT ct: decreased
b. PLT morphology: normal
c. PLT diameter: normal
d. PLT function test: normal
7) None

Question 4

Answer the ff questions:

Given disease: X-linked Thrombocytopenia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 4

1) None
2) X-linked
3) Decreased production of PLTs
4) Mutation in the WAS and GATA1 gene of chromosome X
5) a. Absent or mild bleeding (small PLTs)
b. Severe bleeding (large PLTs)
6) a. PLT ct: decreased
b. PLT diameter: small to large
7) None

Question 5

Answer the ff questions:

Given disease: Acute Immune Thrombocytopenic Purpura

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 5

1) Idiopathic thrombocytopenic purpura
2) None
3) a. Increased PLT destruction
b. Occurs 1 - 3 wks after a viral infection or vaccination (MMR, DTP, polio, hepa A, hepa B)
c. AlloAbs produced against a viral infection or through vaccination binds on PLT surface
4) a. Primarily a disorder of children
b. Seen occasionally in adults
c. Spontaneous remission usually happens
5) a. Abrupt onset of bruising
b. Petechiae
c. Epistaxis
d. Scattered petechiae if mild
e. Extensive petechiae, ecchymoses, hematuria, and epistaxis if acute
f. Gastrointestinal bleeding, hematuria, mucous membrane bleeding, retinal hemorrhage, and intracranial hemorrhage if severe
6) a. PLT ct: decreased -> less than 20,000 / uL
b. No sp test
7) a. Can recover w/out treatment
b. Corticosteroids
c. IVIG
d. Anti-D Ig

Question 6

Answer the ff questions:

Given disease: Chronic Immune Thrombocytopenic Purpura

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 6

1) None
2) None
3) a. Increased PLT destruction
b. IgG autoAbs act against GP IIb and GP IIIa
c. Megakaryocytes are also destroyed
4) a. Primarily a disorder of pts bet 20 - 50 yo
b. Can be occur to any pt of any age
c. Associated w/ HIV infection, hemophilia, pregnancy
d. Spontaneous remission is rare
5) a. Mucocutaneous gradual bleeding
b. Menorrhagia
c. Recurrent epistaxis
d. Easy bruising
e. Ecchymoses
6) a. PLT ct: decreased -> 30,000 - 80,000 / uL
b. PLT diameter: enlarged
c. MPV: increased
7) a. IVIG - treatment of choice
b. Prednisone - initial treatment
c. Corticosteroid
d. Rituximab
e. Splenectomy
f. High-dose dexamethasone or TPO agonist
g. PLT transfusion

Question 7

Answer the ff questions:

Given disease: Neonatal Alloimmune Thrombocytopenia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 7

1) None
2) None
3) a. Increased PLT destruction
b. Maternal alloAbs targeting fetal PLT Ags
c. Mother lacks a PLT-sp Ag the fetus inherited from the father
4) Exposure of mother to paternal Ag will induce the production of alloAbs
5) a. Asymptomatic (affected mother)
b. Petechiae & purpuric hemorrhages (affected fetus)
c. Intracranial hemorrhage
6) a. PLT ct: decreased
b. PLT typing: (+) -> maternal Ab against paternal PLT Ag
7) a. Serial cordocentesis - fetus
b. Intrauterine PLT transfusion - fetus
c. IVIG therapy - fetus
d. Washed maternal PLTs - fetus
e. High-dose corticosteroids - mother

Question 8

Answer the ff questions:

Given disease: Neonatal Autoimmune Thrombocytopenia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 8

1) None
2) None
3) a. Increased PLT destruction
b. Maternal PLT autoAbs due to chronic ITP
c. Maternal PLT autoAbs due to SLE
4) a. Neonates do not have ongoing immune process compared to NAIT
b. AutoAbs are transferred through placenta
5) a. Intracranial hemorrhage
b. Mild bleeding
6) PLT ct: decreased
7) a. Corticosteroid therapy - fetus
b. IVIG treatment - fetus
c. PLT transfusion - fetus
d. Corticosteroid - mother

Question 9

Answer the ff questions:

Given disease: Post Transfusion Purpura

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 9

1) None
2) None
3) a. Increased PLT destruction
b. Develops 1 wk after transfusing PLT-containing blood products
c. Recipient’s plasma alloAbs due to donor’s PLT Ag
4) Common to multiparous middle-aged women
5) Moderate to severe hemorrhage
6) PLT ct: decreased
7) a. Therapeutic exchange transfusion
b. IVIG therapy
c. Corticosteroid therapy

Question 10

Answer the ff questions:

Given disease: Essential Thrombocytopenia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 10

1) Primary thrombocytopenia
2) None
3) a. Increased circulating PLTs
b. Uncontrolled proliferation of marrow megakaryocytes
4) a. Diagnosed by excluding all the causes of reactive or secondary thrombocytosis
b. Prevalent in middle-aged and older pts
c. 1/4 of pt population have a JAK1 and JAK2 mutation
5) a. Thrombosis in the microvasculature - digital pain, digital gangrene, erythromelalgia (throbbing, aching and burning sensation in the extremities, particularly in the palms and soles
b. Thrombosis of large veins and arteries
c. Neurologic complications
d. Hemorrhagic eps
e. Gastrointestinal tract bleeding
f. Bleeding in nose and mouth
g. Urinary tract bleeding
h. Bleeding in skin
6) a. PLT ct: increased -> > than 1,000,000 / uL
b. PLT color: clear or light blue
c. PLT granules: decreased -> hypogranular or agranular
d. Marrow megakaryocytes ct: increased
e. Marrow megakaryocytes size: enlarged
f. Plasma thromboxane A2: increased
g. Plasma beta (sign)-thromboglobulin: increased
h. PLT aggregation response to ADP: decreased
i. PLT aggregation response to collagen: normal
j. PLT aggregation response to epinephrine: absent
k. JAK1 or JAK2 mutation: (+) -> not all the time
7) a. Myelosuppresive agents - melphalan, busulfan
b. Therapeutic plateletpheresis
c. Pegylated interferon-alpha (sign)
d. Anagrelide
e. Low-dose aspirin
f. Hydroxyurea
g. Ruxolitnib - for JAK mutation (+) pts

Question 11

What are the other conditions affecting quantity of PLTs?

Answer 11

1) Bernard-Soulier Syndrome
2) Wiskott-Aldrich Syndrome
3) May-Hegglin Anomaly
4) Sebastian Syndrome
5) Fechtner Syndrome
6) Esptein Syndrome
7) Thrombocytopenia w/ Absent RadII Syndrome
8) Montreal PLT Syndrome
9) Fanconi Anemia
10) Neonatal Non-Immune Thrombocytopenia
11) Megaloblastic Anemia
12) Gestational Thrombocytopenia
13) Hemolyic Disease of Newborn
14) Hemolytic Uremic Syndrome in Children
15) Hemolytic Uremic Syndrome in Adult
16) Atypical Hemolytic Uremic Syndrome
17) Acute Disseminated Intravascular Coagulation
18) Chronic Disseminated Intravascular Coagulation
19) Big Spleen Syndrome
20) Moschcowtiz Syndrome
21) Upshaw-Shulman Syndrome
22) Purpura Fulminans
23) Hypothermia
24) Circulatory Devices in Surgery
25) Massive Transfusion
26) Acute Hemorrhage
27) Major Surgical Procedures
28) Marrow Suppresive Therapy
29) Post-Splenectomy
30) Iron-Deficiency Anemia
31) Kawasaki Disease
32) Exercise-Induced Thrombocytosis

Question 12

Answer the ff questions:

Given disease: Bernard-Soulier Syndrome

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 12

1) Giant PLT syndrome
2) Autosomal recessive
3) a. Decreased production of PLTs
b. Missing GP Ib/IX/V receptor
c. Defective or impaired functionality of GP Ib/IX/V receptor
d. Presence of Abs against GP Ib/IX/V receptor (pseudoBSS)
4) a. Proteins in these complex are made in the ratio of 2:2:2:1 -> GP Ibalpha (sign), GP Ibbeta (sign), GP IX, and GP V
b. Homozygotes have enlarged PLTs, thrombocytopenia, and decreased PLT survival
c. Heterozygotes have normal or near-normal PLT function
5) a. Ecchymoses
b. Epistaxis
c. Gingival bleeding
d. Hemarthroses
e. Hematoma
6) a. PLT aggregation response to ADP: normal
b. PLT aggregation response to arachidonic acid: normal
c. PLT aggregation response to collagen: normal
d. PLT aggregation response to epinephrine: normal
e. PLT aggregation response to ristocetin: absent
f. PLT aggregation response to thrombin: decreased
g. PLT ct: decreased -> until 40,000 / uL
h. PLT diameter: enlarged -> 5 - 8 um to 20 um
i. Cytoplasmic vacuoles and membrane complexes in PLTs: present
j. Irregular demarcation system in megakaryocytes: present
7) a. PLT transfusion - apheresis PLTs or leukoreduced PLTs
b. Desmopressin acetate (DDAVP)
c. Recombinant factor VIIIa

Question 13

Answer the ff questions:

Given disease: Wiskott-Aldrich Syndrome

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 13

1) Classic form is called eczema-thrombocytopenia immunodeficiency syndrome
2) X-linked
3) a. Decreased production of PLTs
b. Dense granule deficiency is 1 of its clinical features
c. Also characterized by increased PLT sequestration
4) None
5) a. Severe eczema
b. Recurrent bacterial, viral, and fungal infections
c. Inability to make polysaccharide Abs -> pneumococcal sepsis
6) a. PLT aggregation response to ADP: decreased
b. PLT aggregation response to collagen: decreased
c. PLT aggregation response to epinephrine: decreased
d. PLT aggregation response to thrombin: normal
e. PLT ct: normal
f. PLT diameter: reduced -> microthrombocytes
7) a. Splenectomy
b. PLT transfusion
c. Bone marrow transplantation

Question 14

Answer the ff questions:

Given disease: May-Hegglin Anomaly

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 14

1) None
2) Autosomal dominant
3) a. Decreased production of PLTs
b. Abnormal microtubule distribution on PLTs
c. Mutation in MYH9 gene that encodes for a cytoskeletal protein in PLT
4) None
5) Mild bleeding
6) a. PLT ct: decreased -> 60,000 / uL - 100,000 / uL
b. PLT diameter: enlarged -> 20 um size
c. Dohle body-like neutrophil inclusions in WBCs: present

Question 15

Answer the ff questions:

Given disease: Sebastian Syndrome

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 15

1) None
2) Autosomal dominant
3) a. Decreased production of PLTs
b. Mutation in MYH9 gene that encodes for a cytoskeletal protein in PLT
4) None
5) a. Mild bleeding
b. Epistaxis
c. Post-operative hemorrhage
6) a. PLT ct: decreased -> 40,000 / uL - 120,000 / uL
b. PLT diameter: enlarged
c. Neutrophil inclusions in WBCs: present

Question 16

Answer the ff questions:

Given disease: Fechtner Syndrome

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 16

1) None
2) Autosomal dominant
3) a. Decreased production of PLTs
b. Mutation in MYH9 gene that encodes for a cytoskeletal protein in PLT
4) None
5) a. Deafness
b. Cataracts
c. Nephritis
6) a. PLT ct: decreased -> 30,000 / uL - 90,000 / uL
b. PLT diameter: enlarged
7) None

Question 17

Answer the ff questions:

Given disease: Esptein Syndrome

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 17

1) None
2) Autosomal dominant
3) a. Decreased production of PLTs
b. Mutation in MYH9 gene that encodes for a cytoskeletal protein in PLT
4) None
5) a. Mild bleeding
b. Epistaxis
c. Gastrointestinal bleeding
d. Female genital tract bleeding
e. Glomerular nephritis
f. High-frequency hearing loss
g. Proteinuria
h. Deafness
i. Ocular problems
6) a. PLT ct: decreased -> 30,000 / uL - 60,000 / uL
b. PLT diameter: enlarged
c. PLT shape: spherical
d. PLT structure: SCCS is prominent
7) None

Question 18

Answer the ff questions:

Given disease: Thrombocytopenia w/ Absent RadII Syndrome

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 18

1) TAR syndrome
2) Autosomal recessive
3) a. Decreased production of PLTs
b. Dense granule defect is 1 of its clinical features
4) None
5) a. Congenital absence extreme hypoplasia of radial bones
b. Cardiac lesions
c. Numerous skeletal abnormalities
d. Transient leukemoid rxns
6) a. PLT aggregation response to ADP: decreased
b. PLT aggregation response to collagen: decreased
c. PLT aggregation response to epinephrine: decreased
d. PLT aggregation response to thrombin: normal
e. PLT ct: decreased -> 10,000 / uL - 30,000 / uL; normal
f. Megakaryocytes: immature, decreased to normal
g. WBC ct: decreased -> > 100,000 / uL
7) None

Question 19

Answer the ff questions:

Given disease: Montreal PLT Syndrome

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 19

1) None
2) Autosomal dominant
3) a. Decreased production of PLTs
b. Defect in regulation of binding site for adhesive proteins
4) None
5) Significant bruising
6) a. PLT ct: decreased -> 5,000 / uL - 40,000 / uL
7) None

Question 20

Answer the ff questions:

Given disease: Fanconi Anemia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 20

1) None
2) Autosomal dominant
3) Decreased production of PLTs
4) None
5) a. Bony abnormalities
b. Pancytopenia
6) a. PLT ct: decreased
b. RBC ct: decreased
c. WBC ct: decreased
7) None

Question 21

Answer the ff questions:

Given disease: Neonatal Non-Immune Thrombocytopenia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 21

1) None
2) None
3) a. Decreased production of PLTs
b. TORCH syndrome is the most common cause
4) a. Toxoplasmosis
b. Other (Treponema pallidum, VZV, parvovirus B19)
c. Rubella
d. Cytomegalovirus
e. Herpes
5) Thrombocytopenia present or within 72 hrs of birth
6) a. PLT ct: decreased -> less than 150,000 / uL
b. PLT diameter: reduced
7) None

Question 22

Answer the ff questions:

Given disease: Megaloblastic Anemia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 22

1) Pernicious anemia
2) None
3) a. Decreased production of PLTs
b. Impaired DNA synthesis
4) Ineffective erythrocyte production goes w/ ineffective PLT production as well
5) Mild bleeding
6) a. PLT ct: decreased -> mild
b. PLT diameter: enlarged
c. Blood folic acid: decreased
d. Blood vitamin B12: decreased
7) 1 - 2 wks vitamin replacement

Question 23

Answer the ff questions:

Given disease: Gestational Thrombocytopenia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 23

1) Incidental thrombocytopenia of pregnancy; pregnancy-associated thrombocytopenia
2) None
3) a. Increased PLT destruction
b. Benign physiologic condition wherein PLT-associated Ig is produced
4) a. Associated w/ hypertensive disorders: preeclampsia, preeclampsia - eclampsia, preeclampsia w/ chronic hypertension, chronic hypertension, gestation hypertension
b. Associated w/ HELLP syndrome - microangiopathic hemolysis, elevated liver enzymes, low PLT ct
c. Associated w/ HIV infection, SLE, TTP, HUS, and antiphospholipid syndrome
5) a. Hypertension - preeclampsia
b. Proteinuria - preeclampsia
c. Abdominal pain - preeclampsia
d. Headache - preeclampsia
e. Blurred vision - preeclampsia
f. Mental function disturbances - preeclampsia
g. Microangiopathic hemolysis - HELLP syndrome
6) PLT ct: decreased -> mild, 100,000 - 150,000 / uL
7) a. No treatment required
b. If w/ preeclampsia, deliver the infant as soon as possible

Question 24

Answer the ff questions:

Given disease: Hemolytic Disease of Newborn

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 24

1) None
2) None
3) a. Increased PLT production
b. PLTs are destroyed upon interacting w/ RC breakdown products
4) None
5) None
6) PLT ct: decreased -> moderate
7) None

Question 25

Answer the ff questions:

Given disease: Hemolytic Uremic Syndrome in Children

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 25

1) None
2) None
3) a. Increased PLT production
b. Shiga toxin, Shiga-like toxin-1 or Shiga-like toxin-2 attach to renal endothelial cells that releases VWF multimers
4) Organisms included Shigella dysenteriae, enterohemorrhagic Escherichia coli O157:H7
5) a. Bloody diarrhea
b. RBC fragmentation
c. Renal failure
d. Proteinuria
6) a. PLT ct: decreased -> mild to moderate
b. BUN: increased
c. Blood crea: increased
d. Blood Hgb: decreased -> < 10 g/dL
e. Reticulocyte ct: increased
f. Peripheral blood film: presence of schistocytes
7) Renal dialysis

Question 26

Answer the ff questions:

Given disease: Hemolytic Uremic Syndrome in Adult

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 26

1) None
2) None
3) a. Increased PLT destruction
b. Immunosuppressive agents or chemotherapeutic agents damage renal endothelial cells that releases VWF multimers
4) Associated w/ postpartum period, pregnancy, oral contraceptive use
5) a. Renal impairment
b. Proteinuria
c. Hemolytic anemia
6) a. PLT ct: decreased -> mild - moderate
b. BUN: increased
c. Blood crea: increased
d. Blood Hgb: decreased -> < 10 g/dL
e. Reticulocyte ct: increased
f. Peripheral blood film: presence of schistocytes
7) Dialysis

Question 27

Answer the ff questions:

Given disease: Atypical Uremic Syndrome (or Atypical Hemolytic Uremic Syndrome?)

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 27

1) aHUS
2) None
3) a. Increased PLT destruction
b. Defect in complement regulatory proteins
c. Complement activation continuously happens and PLT is activated as well
4) None
5) None
6) PLT ct: decreased
7) Eculizumab

Question 28

Answer the ff questions:

Given disease: Acute Disseminated Intravascular Coagulation

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 28

1) None
2) None
3) a. Increased PLT destruction
b. Coagulation cascade is activated by certain agents or conditions
c. PLTs are consumed during the process
4) Formed thrombi are composed of PLTs and fibrinogen
5) Life threatening
6) a. PLT ct: decreased -> severe
b. Factor V: decreased
c. Factor VIII: decreased
d. Factor I: decreased
e. Reticulocyte ct: increased
f. D-dimer: (+)
7) None

Question 29

Answer the ff questions:

Given disease: Chronic Disseminated Intravascular Coagulation

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 29

1) None
2) None
3) a. Increased PLT production
b. Underlying conditions cause low-grade consumptive coagulopathy
4) Can convert into its acute form
5) Not life threatening
6) a. PLT ct: decreased -> severe
b. Factor V: slightly decreased
c. Factor VIII: slightly decreased
d. Factor I: slightly decreased
e. D-dimer: (+)
7) None

Question 30

Answer the ff questions:

Given disease: Big Spleen Syndrome

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 30

1) Splenomegaly
2) None
3) a. Distribution or dilution abnormalities
b. PLTs are physically sequestered
4) a. Associated w/ end-stage liver disease, hepatic cirrhosis, portal hypertension, Hodgkin lymphoma, non-Hodgkin lymphoma, sarcoidosis, chronic myelogenous leukemia, hairy cell leukemia, Gaucher disease
b. Total body PLT ct is still normal
5) None
6) PLT ct: decreased
7) None

Question 31

Answer the ff questions:

Given disease: Moschcowtiz Syndrome

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 31

1) Acquired thrombotic thrombocytopenic purpura
2) None
3) Increased PLT destruction
4) a. Associated w/ viral-like illness days before disease onset
b. Has 4 types - (1) acute TTP, (2) recurrent TTP, (3) drug-induced TTP, and (4) hereditary TTP
5) a. Triad - microangiopathic hemolytic anemia, thrombocytopenia, and neurological abnormalities
b. Pentad - microangiopathic hemolytic anemia, thrombocytopenia, neurological abnormalities, fever and renal dysfunction
c. Diarrhea, anorexia, nausea, weakness, fatigue
6) PLT ct: decreased
7) None

Question 32

Answer the ff questions:

Given disease: Upshaw-Shulman Syndrome

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 32

1) Congenital thrombotic thrombocytopenic purpura
2) Autosomal recessive
3) a. Increased PLT destruction
b. Deficient or dysfunctional ADAMTS13
c. AutoAbs are formed against ADAMTS13
d. Loss of ADAMTS13 results to accumulation of VWF multimers
4) VWF, PLTs and small amt of fibrin and fibrinogen make up the thrombi found in the end arterioles and capillaries
5) a. Hemolysis
b. Neurological disturbances - headache, paresthesia, coma
c. Visual disturbances
d. Renal dysfunction - proteinuria, hematuria, anuria, hgburia
e. Gastrointestinal bleeding
f. Abdominal pain
6) a. PLT ct: decreased
b. Peripheral blood film (nucleated RBCs): present
c. Peripheral blood film (schistocytes): present
d. ADAMTS13: decreased
e. Haptoglobin: decreased
f. Serum unconjugated bili: increased
g. Serum lactate dehydrogenase: increased
h. Partial thromboplastin time: normal
i. Prothrombin time: normal
j. Plasma fibrinogen: normal
k. D-dimer: (-)
7) a. Prophylactic plasma infusion
b. Therapeutic plasma exchange using fresh frozen plasma
c. Corticosteroids
d. Infusions of fresh frozen plasma
e. Vincristine
f. Azathioprine
g. Rituximab
h. Splenectomy
i. PLT transfusions (only if intracranial bleeding is present)
j. Recombinant ADAMTS13

Question 33

Answer the ff questions:

Given disease: Purpura Fulminans

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 33

1) None
2) None
3) a. Increased PLT destruction
b. Bacterial infections brought by Neisseria meningitidis, Streptococcus pneumoniae, grp A and B streptococci, Haemophilus influenzae, Staph aureus
c. Resulting sepsis leads to widespread activation of inflammatory response, coagulation cascade and complement pathway
4) Common to multiparous middle-aged women
5) a. Necrosis of superficial skin
b. Necrosis of deeper soft tissues
c. Gangrenous superficial and deep soft tissues
d. Microvascular necrosis
e. Irregular areas of blue-black cutaneous bleeding
6) a. PLT ct: decreased
b. Partial thromboplastin time: prolonged
c. PT: prolonged
d. Plasma fibrinogen: decreased
e. D-dimer: (+)
7) a. Human-derived PLT concentrate
b. Antithrombotic therapy
c. Coumadin
d. Low-molecular-wt heparin

Question 34

Answer the ff questions:

Given disease: Hypothermia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 34

1) None
2) None
3) a. Distribution or dilution abnormalities
b. Body temp is lowered to < 25 DC
c. Returns to baseline values when body temp is brought back to normal
4) None
5) None
6) PLT ct: decreased -> mild
7) None

Question 35

Answer the ff questions:

Given disease: Circulatory Devices in Surgery

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 35

1) None
2) None
3) a. Distribution or dilution abnormalities
b. PLTs are partially activated inside the pump
4) Associated w/ coronary bypass circuits, ventricular assist devices, extracorporeal membrane oxygenation
5) None
6) PLT ct: decreased -> severe
7) None

Question 36

Answer the ff questions:

Given disease: Massive Transfusion

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 36

1) None
2) None
3) a. Distribution or dilution abnormalities
b. The PLTs of stored blood have impaired viability and function
c. Newly transfused PLTs that are defective are immediately sequestered
4) None
5) None
6) PLT ct: decreased -> severe
7) None

Question 37

Answer the ff questions:

Given disease: Acute Hemorrhage

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 37

1) Acute blood loss
2) None
3) a. Increased circulating PLTs
b. Thrombocytopenia for 2 - 6 days -> thrombocytosis for few days -> normal PLT ct
4) None
5) None
6) PLT ct: increased -> 450,000 - 800,000 /uL
7) None

Question 38

Answer the ff questions:

Given disease: Major Surgical Procedures

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 38

1) None
2) None
3) a. Increased circulating PLTs
b. Normal PLT ct after 10 - 16 days
4) None
5) None
6) PLT ct: increased -> 450,000 - 800,000 /uL
7) None

Question 39

Answer the ff questions:

Given disease: Marrow Suppressive Therapy

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 39

1) Rebound thrombocytosis
2) None
3) a. Increased circulating PLTs
b. Intake of involved drug results to thrombocytopenia; withdrawal of involved drug after medication results to thrombocytosis
c. Rebound peaks at 10 - 17 days after drug withdrawal
4) None
5) None
6) PLT ct: increased
7) None

Question 40

Answer the ff questions:

Given disease: Post-Splenectomy

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 40

1) None
2) None
3) a. Increased circulating PLTs
b. Removal of spleen allows the splenic PLT pool (sequestered PLTs) to join the circulating PLT pool
4) None
5) None
6) PLT ct: increased -> > 1,000,000 /uL
7) None

Question 41

Answer the ff questions:

Given disease: Iron-Deficiency Anemia

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 41

1) None
2) None
3) a. Increased circulating PLTs
b. Iron regulates thrombopoiesis
c. Exact mechanism unknown
4) None
5) None
6) PLT ct: increased -> up to 2,000,000 /uL
7) Iron therapy

Question 42

Answer the ff questions:

Given disease: Kawasaki Disease

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 42

1) Mucocutaneous lymph node syndrome
2) None
3) a. Increased circulating PLTs
b. Inflammation of the walls of small and medium-sized arteries throughout the body
c. Acute febrile stage happens, followed by subacute phase
d. Thrombocytosis happens in subacute phase
4) None
5) a. Mild normochromic, normocytic anemia
b. Cardiovascular complications
c. Aneurysms
6) a. PLT ct: increased -> up to 2,000,000 /uL
b. CRP: increased
c. ESR: increased
d. WBC ct: increased
7) a. Aspirin
b. IVIG

Question 43

Answer the ff questions:

Given disease: Exercise-Induced Thrombocytosis

1) What is the other name?
2) How is it inherited?
3) What are the pathophysiology?
4) What are the characteristics?
5) What are the clinical manifestations?
6) What are the lab studies / results?
7) What is the recommended therapy?

Answer 43

1) None
2) None
3) a. Increased circulating PLTs
b. PLTs are released from spleen during strenous exercise
4) None
5) None
6) PLT ct: increased
7) Completion of exercise - PLT cts return to normal after 30 mins

Question 44

What are the other conditions?

Answer 44

1) Bacterial infections
2) Viral infections
3) Bone marrow infiltration
4) Uncategorized
5) Myeloproliferative neoplasms
6) Inflammation

Question 45

Answer the ff questions:

Given condition: Bacterial infections

1) What are the pathophysiology?
2) What are the clinical significances?

Answer 45

1) Decreased production of PLTs

2) Meningococcemia

Question 46

Answer the ff questions:

Given condition: Viral infections

1) What are the pathophysiology?
2) What are the clinical significances?

Answer 46

1) Decreased production of PLTs
2) a. CMV
b. Varicella-zoster virus
c. Rubella virus
d. Epstein-Barr virus
e. Dengue virus serotypes

Question 47

Answer the ff questions:

Given condition: Bone marrow infiltration

1) What are the pathophysiology?
2) What are the clinical significances?

Answer 47

1) Decreased production of PLTs
2) a. Myeloma
b. Lymphoma
c. Metastatic cancer
d. Myelofibrosis

Question 48

Answer the ff questions:

Given condition: Uncategorized

1) What are the pathophysiology?
2) What are the clinical significances?

Answer 48

1) Distribution or dilution abnormalities
2) a. Chronic renal failure
b. Severe iron deficiency
c. Post-compression sickness
d. Chronic hypoxia

Question 49

Answer the ff questions:

Given condition: Myeloproliferative neoplasms

1) What are the pathophysiology?
2) What are the clinical significances?

Answer 49

1) Increased circulating PLTs
2) a. Polycythemia vera
b. Chronic myelogenous leukemia
c. Myelofibrosis w/ myeloid metaplasia / primary myelofibrosis

Question 50

Answer the ff questions:

Given condition: Inflammation

1) What are the pathophysiology?
2) What are the clinical significances?

Answer 50

1) Increased circulating PLTs
2) a. RA
b. Rheumatic fever
c. Osteomyelitis
d. Ulcerative colitis
e. Acute infections
f. Tumor

Question 51

What are the miscellaneous therapeutic drugs affecting PLT quality (or quantity?)?

Answer 51

1) Drug-dependent AlloAbs
2) Hapten-induced AlloAbs
3) Drug-independent AutoAbs
4) Immune Complex-induced Thrombocytopenia
5) Secondary Thrombocytopenia

Question 52

Answer the ff questions:

Given miscellaneous therapeutic drugs: Drug-dependent AlloAbs

1) What are the involved drugs?
2) What are the pathophysiology?
3) What are the characteristics?
4) What are the clinical manifestations?
5) What are the lab studies / results?
6) What are the recommended therapies?

Answer 52

1) a. Quinine
b. Quinidine
c. Sulfonamide derivatives
2) a. Increased PLT destruction
b. Involved drugs induce alloAbs and target PLTs only in the presence of the drug
c. Abs act against GP Ib/IX/V or GP IIb/IIIa
3) a. IgG class are usually involved
b. IgM class are involved in rare instances
4) Abrupt onset of bleeding symptoms
5) PLT ct: decreased -> < 10,000 /uL
6) a. Identify the offending drug
b. Discontinue drug administration
c. Substitute w/ another therapeutic agent
d. PLT transfusions
e. High-dose IVIG

Question 53

Answer the ff questions:

Given miscellaneous therapeutic drugs: Hapten-induced AlloAbs

1) What are the involved drugs?
2) What are the pathophysiology?
3) What are the characteristics?
4) What are the clinical manifestations?
5) What are the lab studies / results?
6) What are the recommended therapies?

Answer 53

1) a. Penicillin
b. Penicillin derivatives
2) a. Increased PLT destruction
b. Involved drugs act as a hapten and combine w/ a larger molecule to induce alloAbs and target PLTs
c. Abs act against PLT surface
3) Happens if drug molecules are too small
4) a. Severe and rapid bleeding
b. Hemorrhagic bullae in the mouth
5) PLT ct: decreased -> < 1,000 - 10,000 /uL
6) a. Identify the offending drug
b. Discontinue drug administration
c. Substitute w/ another therapeutic agent
d. PLT transfusions
e. High-dose IVIG

Question 54

Answer the ff questions:

Given miscellaneous therapeutic drugs: Drug-independent AlloAbs (or Drug-independent AutoAbs?)

1) What are the involved drugs?
2) What are the pathophysiology?
3) What are the characteristics?
4) What are the clinical manifestations?
5) What are the lab studies / results?
6) What are the recommended therapies?

Answer 54

1) a. Gold salts
b. Procainamide
c. Levodopa
2) a. Increased PLT destruction
b. Involved drugs induce autoAbs and target PLTs even if the drug is not present
c. Abs act against PLT membrane
3) Exact mechanism on autoAb production is unknown
4) Bleeding
5) PLT ct: decreased
6) a. Identify the offending drug
b. Discontinue drug administration
c. Substitute w/ another therapeutic agent
d. PLT transfusions
e. High-dose IVIG

Question 55

Answer the ff questions:

Given miscellaneous therapeutic drugs: Immune Complex-induced Thrombocytopenia

1) What are the involved drugs?
2) What are the pathophysiology?
3) What are the characteristics?
4) What are the clinical manifestations?
5) What are the lab studies / results?
6) What are the recommended therapies?

Answer 55

1) Heparin
2) a. Increased PLT destruction
b. Involved drugs initiate PLT activation and aggregation
3) a. Consumption of PLTs lead to thrombocytopenia
b. Begins 5 - 14 days after drug exposure
4) a. Rarely bleed
b. W/ high risk of thrombosis
5) PLT ct: decreased -> rarely < 15,000 /uL
6) a. Identify the offending drug
b. Discontinue drug administration
c. Substitute w/ another therapeutic agent
d. PLT transfusions
e. High-dose IVIG

Question 56

Answer the ff questions:

Given miscellaneous therapeutic drugs: Secondary Thrombocytopenia

1) What are the involved drugs?
2) What are the pathophysiology?
3) What are the characteristics?
4) What are the clinical manifestations?
5) What are the lab studies / results?
6) What are the recommended therapies?

Answer 56

1) a. Interferons
b. CSF
c. ILs
2) a. Increased PLT destruction
b. Involved biological response modifiers induce IgG that target PLTs
3) Reversible for interferon
4) Bleeding
5) a. PLT ct: decreased -> severe
b. PLT-associated IgG: increased
6) a. Identify the offending drug
b. Discontinue drug administration
c. Substitute w/ another therapeutic agent

Question 57

What is the principle and what are the drugs for sulfonamides?

Answer 57

Principle: maternal ingestion has a cytotoxic effect on fetal marrow
Drugs: chlorothiazide diuretics, tolbutamide oral hypoglycemic medication

Question 58

What is the principle and what are the drugs for chemotherapeutic agents?

Answer 58

Principle: suppress bone marrow production
Drugs: methotrexate, busulfan, cytosine arabinoside, cyclophosphamide, cisplatin

Question 59

What is the principle and what are the drugs for interferon therapy?

Answer 59

Principle: inhibit stem cell differentiation and proliferation
Drugs: interferon-alpha (sign), interferon-gamma (sign)

Question 60

What belong to others - known mechanism?

Answer 60

1) Bone marrow suspension
2) Estrogenic drugs - diethylstilbestrol
3) Antibiotics - chloramphenicol
4) Tranquilizers
5) Anticonvulsants

Question 61

What belong to others - unknown?

Answer 61

1) Affect megakaryopoiesis
2) Zidovudine - HIV treatment
3) Anagrelide
4) Ethanol ingestion

Question 62

What is the principle and drugs for thienopyridine agents?

Answer 62

Principle: induce thrombotic thrombocytopenic purpura
Drugs: ticlopidine, clopidogrel

Question 63

What belong to others?

Answer 63

1) Initiates PLT aggregation and secretion
2) Ristocetin
3) Hematin
4) Protamine sufate
5) Bleomycin