hemoglobin H disease
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, mild to moderate hemolytic anemia
-micro/hypo anemia
-transfusion independent
-hemoglobin H precipitates when oxidized (e.g. by drugs)
leading to hemoglobin H inclusions
-BM shows erythroid hyperplasia
-splenomegaly
qualitative hemoglobinopathy
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gene mutation leads to altered amino acid sequence in the hemoglobin
molecule, affecting the structure and function of the hemoglobin
-amino acids with different charges and biochemical characteristics leads
to dysfunctional and unstable hemolgobin
-e.g. sickle cell disease, hemoglobin C disease, etc.
reversible sickling (SCD)
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poor oxygenation causes RBCs to lose their normal biconcave shape and
become sickle cells
-returning to proper oxygenation allows normal shape to return
-occurs at ≤ 85% O2 in SCD
-occurs at ≤ 40% oxygenation in sickle trait
CD markers
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-hematopoietic stem cells: CD34
-B cells: CD19, CD20
-T cells: CD3
sickle cell trait (heterozygote)
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-patient is mostly asymptomatic
-sickling and crises only occur due to hypoxia (≤40% O2)
-PB is mainly normal, with a few target cells
-Hgb solubility test +
sickle thalassemia (Hgb S-thal)
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combination of hemoglobin S and thalassemia in a patient
-milder anemia compared to SCD alone
-fewer retics than SCD alone
-hemoglobin is decreased, but not as low as SCD alone
gaucher's disease diagnosis
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Give this one a try later!
, mild to moderate hemolytic anemia
-micro/hypo anemia
-transfusion independent
-hemoglobin H precipitates when oxidized (e.g. by drugs)
leading to hemoglobin H inclusions
-BM shows erythroid hyperplasia
-splenomegaly
qualitative hemoglobinopathy
Give this one a try later!
gene mutation leads to altered amino acid sequence in the hemoglobin
molecule, affecting the structure and function of the hemoglobin
-amino acids with different charges and biochemical characteristics leads
to dysfunctional and unstable hemolgobin
-e.g. sickle cell disease, hemoglobin C disease, etc.
reversible sickling (SCD)
Give this one a try later!
poor oxygenation causes RBCs to lose their normal biconcave shape and
become sickle cells
-returning to proper oxygenation allows normal shape to return
-occurs at ≤ 85% O2 in SCD
-occurs at ≤ 40% oxygenation in sickle trait
CD markers
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-hematopoietic stem cells: CD34
-B cells: CD19, CD20
-T cells: CD3
sickle cell trait (heterozygote)
Give this one a try later!
-patient is mostly asymptomatic
-sickling and crises only occur due to hypoxia (≤40% O2)
-PB is mainly normal, with a few target cells
-Hgb solubility test +
sickle thalassemia (Hgb S-thal)
Give this one a try later!
combination of hemoglobin S and thalassemia in a patient
-milder anemia compared to SCD alone
-fewer retics than SCD alone
-hemoglobin is decreased, but not as low as SCD alone
gaucher's disease diagnosis
Give this one a try later!
