PLT, Cancer Composite

What receptor does Bernard Soulier Syndrome lack?

GP 1b

What receptor does Glanzman’s Thrombosthenia lack?

GP 2b/3a

Which syndrome has decreased Ristocetin Aggregation?

Bernard Soulier Syndrome

Which Syndrome has decreased ADP aggregation?

Glanzman’s Thrombasthenia

Which disorder has decreased platelets?

Bernard-Soulier Syndrome

Which disorder has giant platelets?

Bernard-Soulier Syndrome

What PLT function is affected in Bernard-Soulier Syndrome

Adhesion

What platelet function is affected in Glanzman’s Thrombasthenia?

Aggregation

Three features of May-Hegglin Anomaly

1. 1/3 have thrombocytopenia

2. Giant platelets

3. Dohle bodies

What is the gold standard for Heparin-Induced Thrombocytopenia?

Serotonin release assay

What is the easier assay to diagnose HIT?

ELISA for PF4 Ab

What is the pathophysiology of HIT?

Abs form to Heparin-PF4 complex, platelets consume these deposits, leading to clots/thrombosis even though platelets are decreased

What is the pathophysiology of Thrombotic Thrombocytopenic Purpura (TTP)

Pts develop antibody to ADAMTS13, which cleaves vWF. Without this protease, vWF stays ultra large

What three conditions can cause TTP?

1. Pregnancy

2. SLE

3. Viruses

What population is TTP seen in?

30-40 year olds

What are the pentad symptoms of TTP?

1. Thrombocytopenia

2. Hemolytic anemia with RBC fragments

3. Renal abnormalities

4. Fever

5. CNS involvement

What are the triad symptoms of Hemolytic Uremic Syndrome?

1. Renal failure

2. Hemolytic anemia

3. Thrombocytopenia

What causes HUS, and in what population is it usually seen in?

1. E. coli or Shigella infection

2. Kids

Which is more likely to cause CNS involvement: TTP or HUS?

TTP

Which is more likely to cause renal failure: TTP or HUS?

HUS

How does hypergammaglobulinemia prevent clotting?

It coats the vWF factor so platelets can’t adhere to collagen membrane

What test is used to detect hereditary spherocytosis?

Increased osmotic fragility

What is the treatment for hereditary spherocytosis?

Splenectomy

What are three types of crises seen in Hereditary spherocytosis?

1. Hemolytic

2. Aplastic

3. Megaloblastic

What is the pathophysiology of Paroxysmal Nocturnal Hemoglobinuria?

Liver Disease → Acquired mutation of PIGA gene → membrane lipid abnormality → C3b

What two tests are used to detect PNH?

1. Sucrose hemolysis

2. Acid-serum lysis

What is the pathophysiology of G6PD deficiency?

Enzyme defect → H2O2 buildup → HGB denaturation → Heinz body → Splenic pitting

What is Evan’s Syndrome?

WAIHA + Thrombocytopenia

What is seen in CLL?

WAIHA

CAIHA is a post-infectious complication of what oranism?

M. pneumo

Paroxysmal Cold Hemoglobinuria is seen in what population?

Kids with viral infections or lymphomas

What test is used to detect PCH?

Donath Landsteiner Test

What kind of antibody is involved in PCH?

Biphasic antibody

What is the name of AML M0 and a defining feature?

1. Minimally differentiated

2. < 3% blasts are MPO+

What is the name of AML M1 and a defining feature?

1. Without Maturation

2. > 3% blasts are MPO+

Which FAB classification is not in the WHO classification? What is the name of it? What are two visual smear features? What is the effective therapy? What is the genetic abnormality?

1. M3 not in WHO

2. Acute Promyelocytic Leukemia

3. DIC and Hyper/microgranular

4. ATRA

5. t(15;17)

What is the name of AML M4? What is the genetic abnormality? What kind of subtype can be seen?

1. AMMoL

2. inv. 16

3. Eosinophilia subtype

What is the name of AML M5? What is the genetic abnormality?

1. AMoL

2. 11q23

What is the name of AML M6? What is the metric used to measure it?

1. Acute Erythroid Leukemia

2. M:E < 1:1

What is AML M7? What are two features?

1. Acute Megakaryocytic Leukemia

2. MKC’s in PB and Fibrotic marrow

What is ALL L1?

Small blasts

What is ALL L2?

Small and large blasts

What is ALL L3? What is a classic features of these cells? What genetic abnormality is associated with it?

1. Burkitt’s lymphoma

2. Vacuoles

3. t(8;14)

What are two genetic abnormalities for MDS?

Trisomy 8 or 5q-

What are two genetic abnormalities for CLL?

Trisomy 12, 13q-

What genetic abnormality is associated with CML?

t(9;22)