Exam 17: Hemolytic Anemia: Membrane Defects

A patient is suspected of having HS.However,there appear to be <1% spherocytes on the peripheral blood smear,and the osmotic fragility test is normal.What test could be performed to increase the sensitivity of testing for HS?

Differentiate between the three different subtypes of HE based on PB smear morphology.

Predict the results of the following tests in: HS,HE,HPP,PNH,and OHS. a.Ham and sucrose lysis tests b.Osmotic fragility c.Immunophenotyping d.Intracellular ion concentration

Which of the following subtypes of PNH is characterized by moderate hemolysis and intermediate GPI expression?

Which of these disorders has a defective vertical protein interaction between RBC skeleton and the membrane?

A defect in ankyrin is described as:

Which of the following tests is used to screen for PNH?

The characteristic "fish-mouth" central pallor seen in overhydrated hereditary stomatocytosis is caused by an excessive influx of what in the RBCs?

Which of the following disorders is caused by abnormal permeability of the RBC membrane?

Laboratory findings on a stained blood smear exhibit striking erythrocyte morphologic abnormalities.The MCV is decreased,osmotic fragility is abnormal,and the thermal sensitivity test demonstrates an increase in erythrocyte fragmentation.Autohemolysis in increased and is not corrected with glucose.What disorder is associated with these findings?

Defects in integral proteins,protein 4.1,and spectrin are associated with which of the following disorders?

Explain why patients with HS sometimes suffer from gallstones.

Select the best statement from the following choices to describe the pathogenesis of PNH.

Hereditary stomatocytosis is a condition resulting from defects in:

Which of the following disorders is characterized by increased erythrocyte membrane sphingomyelin and decreased lecithin?

Immunophenotyping is performed on a patient suspected of having PNH.What monoclonal antibody specificity should be ordered?

Mary,a 7-year-old,was admitted to the hospital after experiencing severe anemia following a respiratory infection.Her father has a history of RBC membrane abnormality and recently underwent a splenectomy to help alleviate the problem.Upon examination,it was revealed that Mary had hepatosplenomegaly and slight bruising on her forearms.A CBC was ordered,with the following results: HGB 9.0 g/dL HCT 24% WBC 11 × 10⁹/L RBC 3.8 × 10¹²/L MCV 102 fl MCH 25 pg MCHC 40% PLT 275 × 10⁹/L Peripheral smear: moderate macrocytes and stomatocytes Which of the following is most consistent with a presumptive diagnosis in this case?

The function of the MIRL is to:

Which of the following disorders is characterized by both a deficiency in spectrin and the presence of a mutant spectrin protein?

In hereditary spherocytosis,the erythrocytes become trapped in the splenic cords and run out of ATP to pump out excessive ions.What is the ion involved?