Heme Onc

Question 1

Reticulocyte index that indicates BM is responding well

Answer 1

index >2%

Question 2

Packed RBCs facts

Answer 2

no platelets of clotting factors
Mix with normal saline to infuse faster
Each unit raises Hct 3-4 points

Question 3

Fresh frozen plasma indication

Answer 3

Contains all clotting factors

Given for high PT/PTT, coagulopathy, and deficiency of clotting factos

Question 4

Cryoprecipitate indication

Answer 4

Contains factor VIII and fibrinogen

For hemophilia A, decreased fibrinogen (DIC and vWD)

Question 5

Whole blood indication

Answer 5

Massive blood loss

Question 6

Anaphylaxis in Blood transfusion

Answer 6

Within seconds to minutes
Due to ABO mismatched blood products

Symptoms: fever/chills, N/V, pain in flanks/back, chest pain, and dyspnea

Complications: hypovolemic shock, DIC, and renal failure with hemoglobinuria

Treatment: stop trasnfusion immediately, epinephrine, fluid replacement and dopamine/NE

Question 7

Treatment and diagnosis of B thalassemia minor, intermedia and major

Answer 7

Diagnosis
Major: homozygous B chain
Intermedia: indeterminate
Minor: heterozygous B chain
Electrophoresis shows Hb F and Hb A2 are elated
Microcytic hypochromic anemia with target cells

Treatment: frequent packed RBC transfusions are required for major the other two are not treated

Question 8

a -thalassemia trait, silent, Hb H and hydropsy fetalis diagnosis and treatment

Answer 8

diagnosis:
silent: one a locus deletion or mutation
No treatment

Trait: two a loci deletion or mutation
Mild microcytc hypochromic anemia
no treatment

Hb H: 3 loci deleted or mutated
signficant microcytic hypochromic anemia
electrophoresis shows (hemoglobin bart-B tetramers) Hb H
Treatment is packed RBC transfusions and possible splenectomy

hydrops fetalis: all four a loci are mutated or deleted
Fatal at birth

Question 9

Treatment and diagnosis of aplastic anemia

Answer 9

Diagnosis: bone marrow biopsy-hypocellular marrow and absence of progenitor cells of all three cell lines

Treatment: bone marrow transplanation
Transfusion of packed RBCs and platelets if necessary

Question 10

Treatment of B12 deficiency

Answer 10

parenteral cycanocobalamin once per month

Question 11

Treatment of folic acid deficiency

Answer 11

daily oral folic acid replacement

Question 12

General treatment of hemolytic anemia

Answer 12

Transfusion of packed RBCs is severe anemia or patient is hemodynamically unstable
Folate supplements

Question 13

Blood transfusion indications for sickle cell

Answer 13

Acute chest syndrome, stroke, priapism that does not respond to fluids/analgesia, and cardiac decompensation

Question 14

Diagnosis and treatment of hereditary spherocytosis

Answer 14

Diagnosis
Osmotic fragility to hypotonic saline RBCs undergo lysis at higher (thus earlier) oncotic pressure
Elevated reticulocyte count, elevated MCHC

Treatment:
Splenectomy

Question 15

Diagnosis and treatment of warm autoimmune hemolytic anemia

Answer 15

Positive direct coombs test (RBCs coated with IgG)
Splenomegaly
Spherocytes

Treatment: glucocoricoids
Splenectomy
Immunosuppression with azothioprine or cyclophosphamide
RBC transfusions and folate supplements if necessary

Question 16

Diagnosis and treatment of cold autoimmune hemolytic anemia

Answer 16

RBCs coated with complement alone
Avoid cold temps
RBC transfusions if necessary
Various chemotherapeutic agents

Question 17

Diagnosis and treatment of paroxysmal nocturnal hemoglobinuria

Answer 17

Ham’s test: incubated in acidified serum triggering complement pathway-lysis of PNH cells but not normal cells
Sugar water test: patient’s serum mixed in sucrose-hemolysis ensues
Flow cytometery of anchored cell surface proteins-CD55 and CD59-sensitive and specific for PNH

Treatment: glucocorticoids or bone marrow transplantation

Question 18

When to treat heparin induced thrombocytopenia

Answer 18

When thrombocytopenia is seen 3-12 days after heparin induction

Question 19

Diagnosis and treatment of Immune Thrombocytopenic Purpura

Answer 19

Diagnosis: 
No splenomegaly
Platelet count less tan 20,000
Bone marrow has increased megakaryocytes
Increased platelet associated IgG

Treatment:
Adrenal corticosteroids
IV immune globulin: saturates the reticuloenodthelial system
Splenectomy
Romiplastim and eltrombopag: thrombopoietin receptor agonists

Question 20

TTP diagnosis and treatment

Answer 20

Diagnosis:
Prolonged BT, normal PT and PTT
Schistocytes

Treatment:
Plasmapharesis
Corticosteroids and splenectomy may be beneficial
Platelet transfusions are contraindicated

Question 21

Diagnosis and treatment of HIT

Answer 21

Diagnosis: antiplatelet factor IV Ab and serotonin release assay

Treatment: stop heparin
If anticoagulation needed (DVT or PE) use thrombin inhibitor such as lepirudin

Question 22

Diagnosis and treatment of vWF deficiency

Answer 22

Diagnosis:
Prolonged BT-normal platelet count
PTT may be prolonged
Reduced ristoceitin induced platelet aggregation

Treatment: desmorpressi-induces cells to secrete vWF (first line)
Factor VIII concnetrates-after major trauma or during surgery

NO cyroprecipiate or asprin/NSAIDs

Question 23

Treatment of hemophilia A

Answer 23

Acute hemarthorsis:
analgesia with codeine +/- acetaminophen
ice and rest the joint

Factor VIII concentrate-acute bleeding, before surgery and dental surgeries

NOT cryoprecipitate or FFP due to viral transmission risk

Desmopressin may be helpful

Synovesctomy or radiosynovectomy may be needed if severe recurrent hemarthorsis

Mix with normal plasma and if PTT returns to normal then there is not presence of a factor VIII inhibitor

Question 24

Diagnosis and treatment of DIC

Answer 24

Diagnosis
PT, PTT, BT and thrombin time are elevated
Fibrin split products and D dimer

Fibrinogen is low, thrombocytopenia
Schistocytes

Treatment:
FFP replaces clotting factors
Platelet transfusions
Cryoprecipitate replaces clotting factors and fibrinogen

Oxygen, IV fluids, Maintain BP and renal perfusion

Question 25

Hb Indication level for blood transfusion

Answer 25

Less than 7