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20 Cards in this Set

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  • Back
81. best initial therapy for autoimmune hemolysis?
a. Steroids, such as prednisone.
b. If the case describes recurrent episodes of hemolysis, splenectomy is the most effective therapy.
82. CCS tip: if the case describes severe hemolysis not responsive to prednisone or repeated blood transfusion,what should be used?
a. Intravenous immunoglobulins as the best therapy to stop acute episodes of hemolysis.
83. Cold-induced hemolysis (cold agglutinins)- presentation? Look for the following
mycoplasma or Epstein-Barr virus in history.
b. Standard Coombs test is negative!!!
c. Compliment test is positive
d. there is no response to steroids, splenectomy, or IVIG.
84. Treatment of cold-induced hemolysis (cold agglutinins)?
a. Rituximab
85. Presentation of glucose-6 phosphate dehydrogenase deficiency (G6PD)?
a. Sudden onset of hemolysis, which can be quite severe, is seen.
b. As an X-linked disorder, hemolysis from G6PD deficiency is much more often described in males.
86. What is the most common form of oxidant stress to cause acute hemolysis with G6PD deficiency?
a. Infection!
87. Oxidizing drugs which can cause acute analysis and G6PD deficiency?
1. Sulfa medications
2. Primaquine
3. Dapsone.
b. One of these are frequently in the history.
c. Fava bean ingestion may also be in the history.
88. Best initial diagnostic test for G6PD deficiency?
a. Heinz body test, bite cells
b. Heinz bodies are collections of oxidized, precipitated hemoglobin embedded in the red cell membrane.
c. Bite cells appear when pieces of the red cell membrane have been removed for displaying.
89. Most accurate test for G6PD deficiency (glucose six phosphate dehydrogenase)?
a. If the level of G6PD-but ONLY after two months have passed.
b. A normal level of G6PD immediately after episode of hemolysis does not exclude G6PD deficiency.
c. On the day of the hemolysis, the most efficient cells have been destroyed, any level of G6PD is normal.
90. Treatment of glucose-6 phosphate dehydrogenase deficiency?
a. There is no specific therapy.
b. Avoid oxidant stress.
91. Presentation of pyruvate kinase deficiency?
a. Presents the same way as G6PD deficiency in terms of hemolysis.
b. However, pyruvate kinase deficiency is not provoked by medication or or fava beans.
c. Precipitates the small assist with Pyruvate kinase deficiency is not clear.
92. Presentation of hereditary spheroctysis?
a. Recurrent episodes of hemolysis
b. splenomegaly
c. bilirubin gallstones
d. Elevated mean corpuscular hemoglobin concentration (MCHC)
93. diagnostic testing for hereditary spherocytosis?
a. The most accurate diagnostic testis an osmotic fragility test.
94. Treatment of hereditary spherocytosis?
a. Splenectomy will prevent hemolysis, since the cells are destroyed in the spleen.
b. Splenectomy helps these patients.
95. Presentation of hemolytic uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP)?
a. Look for E. coli 0157:87 in the history for HUS.
b. Medications such as Ticlopidine predisposed to TTP.
96. Diagnostic testing for HUS and TTP?
a. There is no specific diagnostic test for these.Rather, the diagnosis is based on the following.
97. HUS triad?
a. ART:
1. intravascular hemolysis with abnormal smear (A: autoimmune hemolysis)
2. elevated BUN and creatinine (R: renal failure)
3. thrombocytopenia (T: Thrombocytopenia)
98. TTP and pentad (FAT RN) also has the following two issues?
a. Fever
b. neurological abnormalities
99. treatment of HUS and TTP?
a. Most cases resolve on their own.
b. Severe cases of either should be treated with plasmapheresis.
c. Steroids will not help.
d. Antibiotics for HUS from E. coli may make it worse.
e. Platelet transfusions for either will definitely make it worse.
100. Note: NEVER use platelets in HUS or TTP.
Note: NEVER use platelets in HUS or TTP.