Role of Haptoglobin
- binds to free hemoglobin to reduce its oxidative toxicity
- a low haptoglobin level is seen in hemolytic anemia
List the causes of macrocytic anemia
- vitamin B 12 deficiency
- Folate deficiency
What is the most common cause of vitamin B12 deficiency
- pernicious anemia : malabsorption
A (+) schilling test is indicative of
pernicious anemia
List the most common etiologies of microcytic anemia
- iron deficiency
- alpha/beta thalassemia
- lead poisoning
What would you expect serum Fe, ferritin, and TIBC levels to be in iron deficiency anemia
- serum Fe: low
- Ferritin: Low
- TIBC: high
What are the normal types of hemoglobin
- adult hemoglobin: make up 95% after age 6 mo.
- HbgA (2 alpha, 2 beta)
- HbgA2 (2 alpha, 2 delta)
- Fetal hemoglobin: HbgF (aa/yy)
What is Cooley’s anemia
- Beta thalassemia major
- both Beta genes are mutated
- patients normal at birth due to fetal hemoglobin but become symptomatic at 6 months
Microcyctic anemia with basophilic stippling is consistent with what condition
lead poisoning
Anemia of chronic disease usually presents with what ferritin level
- increased ferritin (as an acute phase reactant)
G6PD deficiency is what type of genetic disorder? It primarily affects what patient population?
- x-linked recessive
- african americans
Episodic no immune acute hemolytic anemia that presents with Heinz bodies on peripheral smear
G6PD deficiency
Howell-jolly bodies are seen in what condition
sickle cell disease
Children with sickle cell should be immunized against
- S. Pneumococcus
- H. Influenzae type B
- N. Meningococcus
Thrombotic Thrombocytopenic Purpura is caused by
- antibodies against ADAMTS13
- ADAMTS13 is responsible for cutting vWF -> large vWF multi eras adhere to platelets and cause endothelium platelet adhesion -> small vessel thrombosis -> RBC’s become sheared as they circulate in the partially occluded small vessels -> hemolytic anemia
TX of TTP
Plasmapharesis: removes antibodies against ADAMTS13
What is the triad seen in hemolytic uremic syndrome
- microangiopathic hemolytic anemia
- kidney failure
- Thrombocytopenia
hemolytic uremic syndrome is usually preceded by infection with
- E-Coli O157:H7
What is idiopathic thrombocytes purpura
- Autoimmune, antibody against platelets
- MC seen after viral infection
What is Hemophilia A
- Factor VIII deficiency -> affects intrinsic pathway
- x-linked
Hemophilia A most commonly presents with what symptom
- hemarthrosis: bleeding in weight bearing joints (ex: ankles)
What is hemophilia B
- factor IX deficiency
- Christmas disease
- X-linked
Function of vWF
- necessary for initial platelet adhesion
- prevents Factor VIII degradation
Leukemia is the malignancy of what type of cells?
- malignancy of WBC -> bone marrow proliferation of abnormally large amounts of WBCs
- Get pancytopenia: neutropenia, anemia, and thrombocytopenia
