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    Hemoglobinopathies

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    Katerina Bagashvili
    A 34-week stillborn infant from Vietnam presented with hepatosplenomegaly, cardiomegaly, serous effusions, and generalized hydrops without anomalies. Given the findings, hemoglobin Bart's would be the most predominant type found on hemoglobin electrophoresis. A 23-year old Italian man developed hemolytic anemia after eating beans and pasta. Urinalysis showed free hemoglobin and blood smear showed Heinz bodies. Given his symptoms and triggers, G6PD deficiency is the most likely diagnosis. The first line treatment for mild warm antibody autoimmune hemolytic anemia is prednisone 1mg/kg.

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    Hemoglobinopathies

    Uploaded by

    Katerina Bagashvili
    16 views2 pages
    A 34-week stillborn infant from Vietnam presented with hepatosplenomegaly, cardiomegaly, serous effusions, and generalized hydrops without anomalies. Given the findings, hemoglobin Bart's would be the most predominant type found on hemoglobin electrophoresis. A 23-year old Italian man developed hemolytic anemia after eating beans and pasta. Urinalysis showed free hemoglobin and blood smear showed Heinz bodies. Given his symptoms and triggers, G6PD deficiency is the most likely diagnosis. The first line treatment for mild warm antibody autoimmune hemolytic anemia is prednisone 1mg/kg.

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    hemoglobinopathies

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    A 34-week stillborn infant from Vietnam presented with hepatosplenomegaly, cardiomegaly, serous effusions, and generalized hydrops without anomalies. Given the findings, hemoglobin Bart's would be the most predominant type found on hemoglobin electrophoresis. A 23-year old Italian man developed hemolytic anemia after eating beans and pasta. Urinalysis showed free hemoglobin and blood smear showed Heinz bodies. Given his symptoms and triggers, G6PD deficiency is the most likely diagnosis. The first line treatment for mild warm antibody autoimmune hemolytic anemia is prednisone 1mg/kg.

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    © All Rights Reserved
    Download as DOCX, PDF, TXT or read online from Scribd
    Download as docx, pdf, or txt
    16 views2 pages

    Hemoglobinopathies

    Uploaded by

    Katerina Bagashvili
    A 34-week stillborn infant from Vietnam presented with hepatosplenomegaly, cardiomegaly, serous effusions, and generalized hydrops without anomalies. Given the findings, hemoglobin Bart's would be the most predominant type found on hemoglobin electrophoresis. A 23-year old Italian man developed hemolytic anemia after eating beans and pasta. Urinalysis showed free hemoglobin and blood smear showed Heinz bodies. Given his symptoms and triggers, G6PD deficiency is the most likely diagnosis. The first line treatment for mild warm antibody autoimmune hemolytic anemia is prednisone 1mg/kg.

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    © All Rights Reserved
    Download as DOCX, PDF, TXT or read online from Scribd
    Download as docx, pdf, or txt
    of 2

    1.

    A 32-year-old woman from Hanoi, Vietnam, gives birth at 34 weeks’ gestation to a


    markedly hydropic stillborn male infant. Autopsy findings include hepatosplenomegaly
    and cardiomegaly, serous effusions in all body cavities, and generalized hydrops. No
    congenital anomalies are noted. There is marked extramedullary hematopoiesis in visceral
    organs. Which of the following hemoglobins is most likely predominant on hemoglobin
    electrophoresis of the fetal RBCs?
    A. Hemoglobin A1
    B. Hemoglobin A2
    C. Hemoglobin H
    D. Hemoglobin F
    E. Hemoglobin Bart's
    F. Hemoglobin S
    G. Hemoglobin E

    2. A 23-year-old, previously healthy man of Italian origin develops moderate to severe


    hemolytic anemia. The previous evening he had celebrated a Saint’s day with a feast of
    beans and pasta. Urinalysis shows free hemoglobin, and the
    direct Coombs test is negative. Supravital staining of the blood smear demonstrates
    numerous membrane-bound inclusions (Heinz bodies) within erythrocytes. Which of the
    following is the most likely diagnosis?
    A. G6DP deficiency
    B. Paroxysmal nocturnal hemoglobinuria
    C. Sickle cell anemia
    D. β-Thalassemia minor
    E. Warm antibody autoimmune hemolytic anemia

    3. What is the first line treatment of mild Warm Antibody autoimmune hemolytic anemia
    a. Rituximab
    b. Prednisone 1mg/kg
    c. Hematopoietic stem cell transplantation
    d. Azathioprine
    e. Blood transfusion
    f. Splenectomy

    4. Which of the following regarding the Paroxysmal Nocturnal Hemoglobinuria is not true?
    a. PNH is a rare, congenital cause of anemia
    b. There is a mutation in PIG-A gene of the bone marrow stem cells
    c. In PNH red blood cells are susceptible to destruction via activated
    Complement
    d. CD55 and CD59 on the surface of RBCs are deficient
    e. Treatment option includes Eculizumab - anti C5 medication

    5. 5 year-old boy is brought to the clinic with severe acute abdominal pain. Physical exam is
    remarkable for splenomegaly and complete blood count shows marked anemia. What
    morphological change would you expect to find in this patient's peripheral blood?
    a. Increased numbers of spherocytes
    b. Target cells
    c. Bite cells
    d. Sickle cells
    e. Eliptocytes

    6. Which of the following regarding the sickle cell disease treatment is correct?
    a. Pain medications are not indicated for management of painful crisis
    b. Intravenous redydration is shown to increase the duration of patient's
    syndromes
    c. Immunizations are only needed if you plan to do the splenectomy
    d. Hydroxyurea will increase HbS and thus help with patient's symptoms
    e. Folic acid supplementation is needed for these patients

    7. A 10-year-old boy presents with chronic fatigue. Physical examination reveals slight
    jaundice and splenomegaly. The results of laboratory studies include hemoglobin of 11.7
    g/dL, hematocrit of 32%, total bilirubin of 2.6 mg/dL, and conjugated bilirubin of 0.8
    mg/dL. The peripheral blood smear is shown in the image. The osmotic fragility of the
    patient’s RBCs is increased, but the Coombs test is negative. Defects in which of the
    following are involved in the pathogenesis of this disorder?

    A. DNA synthesis
    B. Erythrocyte cytoskeleton
    C. Erythrocyte maturation
    D. Glucose-6-phosphate dehydrogenase (G6PD)
    E. Hemoglobin synthesis

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