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DIT Quizzes Day 15.3
Heme
46
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Professional
05/30/2013

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Term
What allows RBCs to change shape as they pass through vessel?
Definition
Spectrin (accounts for the flexibility and biconcave shape)
Term
What are some of the different causes of polycythemia?
Definition
Excess erythropoeitin: Renal cell carcinoma, pheochromocytoma, hemangioblastoma, chronic hypoxia

Polycythemia vera
Down syndrome (65% at birth)
Term
What coagulation factor is deficient in Hemophilia A?
Definition
VIII
Term
What coagulation factor is deficient in hemophilia B?
Definition
IX
Term
What clotting factors require vitamin K for synthesis?
Definition
X, IX, VII, II, Protein C, Protein S
Term
What are the tx for OD of heparin and warfarin?
Definition
Hep: protamine sulfate

Warfarin: vitamin K or fresh frozen plasma
Term
What lab value is used to monitor the following:
Heparin
Warfarin
enoxaparin
Definition
Heparin: PTT
Warfarin: PT, INR(2-3)
enoxaparin: Factor Xa activity
Term
What is the tx for heparin induced thrombocytopenia
Definition
Anticoagulate with Lepirudin or bivalirudin
Term
What pathologic RBC form would you see in the following disease:
Lead poisoning
Definition
Basophilic stippling
Term
What pathologic RBC form would you see in the following disease:
G6PD deficiency
Definition
Heinz bodies with bite cells
Term
What pathologic RBC form would you see in the following disease:
DIC
Definition
Schistocytes
Term
What pathologic RBC form would you see in the following disease:
Abetalipoproteinemia
Definition
Acanthocyte (spur cell)
Term
What pathologic RBC form would you see in the following disease:
Asplenia
Definition
Howell-Jolly bodies
Term
What is the cause of anemia given the following statements:
Microcytic anemia + swallowing difficulty + glossitis
Definition
Iron deficiency; Plummer-Vinson syndrome
Term
What is the cause of anemia given the following statements:
Microcytic anemia + >3.5 HbA2
Definition
B-thalassemia minor
Term
What is the cause of anemia given the following statements:
Megaloblastic anemia not correctable by B12 or Folate
Definition
Orotic aciduria
Term
What is the cause of anemia given the following statements:
Megaloblastic anemia along with peripheral neuropathy
Definition
B12 deficiency
Term
What is the cause of anemia given the following statements:
Microcytic anemia + basophilic stippling
Definition
Lead poisoning
ACD
B-thalassemia
Iron deficiency anemia
Term
What is the cause of anemia given the following statements:
microcytic anemia reversible with B6
Definition
sideroblastic anemia
Term
What is the cause of anemia given the following statements:
HIV positive patient with macrocytic anemia
Definition
Zidovidine side effect
Term
What is the cause of anemia given the following statements:
Normocytic anemia + red urine in the morning
Definition
Paroxysmal Nocturnal Hemoglobinuria
Term
What is the cause of anemia given the following statements:
Normocytic anemia and elevated creatinine
Definition
Chronic kidney disease
Term
What is the rate limiting step in heme synthesis?
Definition
d-aminolevulinic synthase
Term
What is the cause of ITP?
Definition
anti-GpIIb/IIIa antibodies (platelet destruction)
Term
What is the defect in Bernard-Soulier disease?
Definition
deficiency GpIb (defect in platelet-collagen adhesion)
Term
What is the most common inherited bleeding disorder
Definition
von Willebrand's disease
Term
What are some of the hereditary syndromes of thrombosis
Definition
Factor V Leiden (most common)
Prothrombin gene mutation
Antithrombin
Protein C or S deficiency
Term
Hematologic tests/ findings:
Ham's test
Definition
Paroxysmal nocturnal hematuria
Term
Hematologic tests/ findings:
DEB test
Definition
Fanconi's anemia
Term
Hematologic tests/ findings:
Heinz bodies
Definition
G6PD deficiency
Term
Hematologic tests/ findings:
D-dimers
Definition
DVT, DIC
Term
Hematologic tests/ findings:
Basophilic stippling
Definition
Lead poisoning
Term
Hematologic tests/ findings:
Osmotic fragility test
Definition
Hereditary spherocytosis
Term
Hematologic tests/ findings:
+ ristocetin test
Definition
von Willebrand's disease
Term
What is the most common type of nonHodgkins lymphoma in adults? Children?
Definition
Adults: Diffuse large B cell
Children: Lymphoblastic
Term
What lab findings are indicative of DIC?
Definition
elevated D-dimer
elevated PTT
Elevated bleeding time
Term
What is the structure of HbH?
What disease results?
Definition
Beta4
alpha thalassemia (severe)
Term
What is the structure of Hb Barts?
Definition
no alpha chains
gamma4
Term
What disease results in Hb Barts production?
Definition
Hydrops fetalis
Severe a-thalassemia = death
Term
What are the causes of aplastic anemia
Definition
Radiation
Benzene
Viruses
immune related
fanconi's anemia
Term
In what hematologic disease would you expect to see positive Coomb's test
Definition
Autoimmune hemolytic anemia
Term
What findings are a/w hereditary spherocytosis
Definition
decreased spectrin
positive osmotic fragility test
Howell-Jolly bodies (after splenectomy)
Coomb's negative
Pigmented gallstones
jaundice
Term
A child who had been anemic since birth has been cured via splenectomy. What was the disease?
Definition
Hereditary spherocytosis
Term
What neoplasms are a/w AIDS
Definition
Kaposi sarcoma
Invasive squamous cell carcinoma
CNS lymphoma
NonHodgkins lymphoma
Term
A heart failure patient is newly diagnosed with cancer and is being evaluated for chemo. What chemotherapeutic agent should be avoided in this patient?
Definition
Doxorubicin
Term
Chromosomal analysis of an anemia patient reveals Philadelphia chromosome (t9;22). Treatment?
Definition
Dx CML
Tx Imatinab
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