CPHON CORRECT STUDY GUIDE QUESTIONS AND
ANSWERS SURE A+
✔✔What does Hydroxyurea do? - ✔✔^ Fetal Hgb, vWBC, plt, retics. Myelosuppression.
Indicated in SCD with strong history of acute chest
✔✔What is Exjade for? - ✔✔Treats Iron Toxicity
✔✔How Thalassemia gets transmitted genetically? - ✔✔B-Thal= autosomal recessive
✔✔Factor 8 - ✔✔Treats Hemophilia A. Replace QOD or with bleeding episode
✔✔Factor 9 - ✔✔Treats Hemophilia B. Replace 2-3 times/week
✔✔Autoimmune Hemolytic Anemia (AIHA) (Definition) - ✔✔Antibodies againsts
antigens on RBCs -->Hemolysis
✔✔Autoimmune Hemolytic Anemia (AIHA) (Treatment) - ✔✔Steroids, IVIG (only if
severe), Folic Acid, Splenectomy
✔✔Sickle Cell Disease - ✔✔Hgb A absent and Hgb S present
✔✔Thalassemia (Definition) - ✔✔Genes required to make hgb a are abnormal.
Requires 4 alphas and 2 betas. Beta Thalassemia worse
✔✔Thalassemia (Treatment) - ✔✔Transfusions (major), folic acid, splenectomy,
hydroxyurea, BMT only cure (major)
✔✔G6PD Deficiency - ✔✔Enzyme Defect causes hemolysis upon exposure to various
agents (no fava beans)
, ✔✔Hereditary Spherocytosis (Definition) - ✔✔Hemolytic anemia involving cell
membrane alterations resulting in fragile RBCs trapped in spleen. Shaped like a
Sphere, smaller, and more rigid.
✔✔Hereditary Spherocytosis (Treatment) - ✔✔Epogen, (blood transfusions rare),
Splenectomy in >6yo with severe diagnosis, folic acid
✔✔Aplastic Anemia (Definition) - ✔✔Pancytopenia. Includes Fanconis (can develop in
MDS or AML). X-rays and CTs only when necessary as it causes chromo breaks (MRI
instead). Dx by DEBOR MMC
✔✔Aplastic Anemia (Treatment) - ✔✔SCT is only cure. Preparative regimen(to
immunosuppress): ATG, Cyclosporine, Methylprednisolone, and GCSF
✔✔Dyskeratosis Congenita (DKC) - ✔✔Rare. Decreased telomerase activity leading to
chromo instability. Derm symptoms (triad): 1. skin hyperpigmentation. 2. Nail dystrophy.
3. Leukoplakia
✔✔Diamond Blackfan Anemia - ✔✔Pure red cell aplasia- bone marrow does not
produce RBCs
✔✔Shwachman Diamond Syndrome - ✔✔Rare autorecessive. Pancreatic insufficiency,
FTT, skeletal abnormalities, and BM dysfunction
✔✔Evans Syndrome - ✔✔Combo of AIHA and ITP. Simultaneous or sequential.
✔✔Von Wilebrand - ✔✔Defect of VW Factor. Give VW Factor containing FVIII
✔✔Idiopathic thrombocytopenic purpura - ✔✔Immune System produces antibodies
against platelets. Treat with Steroids, Whin Rho, IVIG, and 6MP. Do not give NSAIDS &
Warfarin
✔✔Paroxysmal nocturnal hemoglobinuria (PNH) - ✔✔Rare life threatening combo of
hemolytic anemia, pancytopenia, and thrombosis
✔✔Early Effects of Peg - ✔✔Anaphylaxis, pancreatitis, coagulopathy
✔✔Early Effects of MTX - ✔✔Mucocitis, photosensitivity, Nephrotoxic (leucovorin and
bicarb), skin burns, AMS (with high doses), do not take folic acid or septra.
✔✔Early Effects of ARA-C - ✔✔Conjunctivitis with eyedrops and n/v, fever, AMS (with
high doses), myelosuppression
ANSWERS SURE A+
✔✔What does Hydroxyurea do? - ✔✔^ Fetal Hgb, vWBC, plt, retics. Myelosuppression.
Indicated in SCD with strong history of acute chest
✔✔What is Exjade for? - ✔✔Treats Iron Toxicity
✔✔How Thalassemia gets transmitted genetically? - ✔✔B-Thal= autosomal recessive
✔✔Factor 8 - ✔✔Treats Hemophilia A. Replace QOD or with bleeding episode
✔✔Factor 9 - ✔✔Treats Hemophilia B. Replace 2-3 times/week
✔✔Autoimmune Hemolytic Anemia (AIHA) (Definition) - ✔✔Antibodies againsts
antigens on RBCs -->Hemolysis
✔✔Autoimmune Hemolytic Anemia (AIHA) (Treatment) - ✔✔Steroids, IVIG (only if
severe), Folic Acid, Splenectomy
✔✔Sickle Cell Disease - ✔✔Hgb A absent and Hgb S present
✔✔Thalassemia (Definition) - ✔✔Genes required to make hgb a are abnormal.
Requires 4 alphas and 2 betas. Beta Thalassemia worse
✔✔Thalassemia (Treatment) - ✔✔Transfusions (major), folic acid, splenectomy,
hydroxyurea, BMT only cure (major)
✔✔G6PD Deficiency - ✔✔Enzyme Defect causes hemolysis upon exposure to various
agents (no fava beans)
, ✔✔Hereditary Spherocytosis (Definition) - ✔✔Hemolytic anemia involving cell
membrane alterations resulting in fragile RBCs trapped in spleen. Shaped like a
Sphere, smaller, and more rigid.
✔✔Hereditary Spherocytosis (Treatment) - ✔✔Epogen, (blood transfusions rare),
Splenectomy in >6yo with severe diagnosis, folic acid
✔✔Aplastic Anemia (Definition) - ✔✔Pancytopenia. Includes Fanconis (can develop in
MDS or AML). X-rays and CTs only when necessary as it causes chromo breaks (MRI
instead). Dx by DEBOR MMC
✔✔Aplastic Anemia (Treatment) - ✔✔SCT is only cure. Preparative regimen(to
immunosuppress): ATG, Cyclosporine, Methylprednisolone, and GCSF
✔✔Dyskeratosis Congenita (DKC) - ✔✔Rare. Decreased telomerase activity leading to
chromo instability. Derm symptoms (triad): 1. skin hyperpigmentation. 2. Nail dystrophy.
3. Leukoplakia
✔✔Diamond Blackfan Anemia - ✔✔Pure red cell aplasia- bone marrow does not
produce RBCs
✔✔Shwachman Diamond Syndrome - ✔✔Rare autorecessive. Pancreatic insufficiency,
FTT, skeletal abnormalities, and BM dysfunction
✔✔Evans Syndrome - ✔✔Combo of AIHA and ITP. Simultaneous or sequential.
✔✔Von Wilebrand - ✔✔Defect of VW Factor. Give VW Factor containing FVIII
✔✔Idiopathic thrombocytopenic purpura - ✔✔Immune System produces antibodies
against platelets. Treat with Steroids, Whin Rho, IVIG, and 6MP. Do not give NSAIDS &
Warfarin
✔✔Paroxysmal nocturnal hemoglobinuria (PNH) - ✔✔Rare life threatening combo of
hemolytic anemia, pancytopenia, and thrombosis
✔✔Early Effects of Peg - ✔✔Anaphylaxis, pancreatitis, coagulopathy
✔✔Early Effects of MTX - ✔✔Mucocitis, photosensitivity, Nephrotoxic (leucovorin and
bicarb), skin burns, AMS (with high doses), do not take folic acid or septra.
✔✔Early Effects of ARA-C - ✔✔Conjunctivitis with eyedrops and n/v, fever, AMS (with
high doses), myelosuppression
