Question 1

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

Accepted Answer

A) Hereditary elliptocytosis 
B) Hereditary pyropoikilocytosis 
C) Hereditary spherocytosis 
D) Acanthocytosis 
A) Hereditary elliptocytosis 
B) Hereditary pyropoikilocytosis 
C) Hereditary spherocytosis 
D) Acanthocytosis C

Question 2

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: 
$\begin{array}{ll}
\text { HGB } & 9.0 \mathrm{~g} / \mathrm{dL} \
\text { HCT } & 24 \% \
\text { WBC } & 11 \times 10^{9} / \mathrm{L} \
\text { RBC } & 3.8 \times 10^{12} / \mathrm{L} \
\text { MCV } & 102 \mathrm{fl} \
\text { MCH } & 25 \mathrm{pg} \
\text { MCHC } & 40 \% \
\text { PLT } & 275 \times 10^{9} / \mathrm{L}
\end{array}$
Peripheral smear: moderate macrocytes and stomatocytes
Which of the following is most consistent with a presumptive diagnosis in this case?

Accepted Answer

A) PNH 
B) HS 
C) DHS 
D) HE 
A) PNH 
B) HS 
C) DHS 
D) HE B

Question 3

The function of the MIRL is to:

Accepted Answer

A) Induce red cell agglutination. 
B) Prevent activation of C3b converting enzyme. 
C) Interfere with C8/C9 activation. 
D) All of the above. 
A) Induce red cell agglutination. 
B) Prevent activation of C3b converting enzyme. 
C) Interfere with C8/C9 activation. 
D) All of the above. C

Question 4

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

Accepted Answer

Ham and sucrose lysis tests: PNH: increa

Question 5

Explain why patients with HS typically have an MCHC greater than 36%.

Accepted Answer

Spherocytes have decreased sur

Question 6

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

Accepted Answer

A) Hereditary elliptocytosis 
B) Hereditary spherocytosis 
C) PNH 
D) Hereditary dehydrated stomatocytosis 
A) Hereditary elliptocytosis 
B) Hereditary spherocytosis 
C) PNH 
D) Hereditary dehydrated stomatocytosis

Question 7

In PNH, cells are susceptible to complement induced lysis.This is thought to be due to the lack of what two regulating factors?

Accepted Answer

A) LCAT and lipoprotein 
B) DAF and MIRL 
C) HDL and VLDL 
D) Beta and alpha lipoprotein 
A) LCAT and lipoprotein 
B) DAF and MIRL 
C) HDL and VLDL 
D) Beta and alpha lipoprotein

Question 8

A defect in ankyrin is described as:

Accepted Answer

A) A vertical interaction defect. 
B) A horizontal interaction defect. 
C) Both vertical and horizontal interaction defects. 
D) It cannot be determined. 
A) A vertical interaction defect. 
B) A horizontal interaction defect. 
C) Both vertical and horizontal interaction defects. 
D) It cannot be determined.

Question 9

Hereditary stomatocytosis is a condition resulting from defects in:

Accepted Answer

A) Cation permeability. 
B) Spectrin. 
C) Protein 4.1. 
D) Spectrin dimer-dimer association. 
A) Cation permeability. 
B) Spectrin. 
C) Protein 4.1. 
D) Spectrin dimer-dimer association.

Question 10

PNH is currently detected in the lab by which of the following?

Accepted Answer

A) Immunophenotyping 
B) Ham and sucrose lysis tests 
C) Donath-Landsteiner test 
D) Osmotic fragility 
A) Immunophenotyping 
B) Ham and sucrose lysis tests 
C) Donath-Landsteiner test 
D) Osmotic fragility

Question 11

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

Accepted Answer

Common HE: predominantly ellip

Question 12

A sample has the following test results: hemoglobin 9 g/dL; autohemolysis is increased after 48 hours; increased hemolysis is observed with the Ham test and sucrose hemolysis test.Based on these findings, what is the patient most likely suffering from?

Accepted Answer

A) HS 
B) HE 
C) PNH 
D) PCH 
A) HS 
B) HE 
C) PNH 
D) PCH

Question 13

What disorder has an abnormal erythrocyte membrane that is abnormally permeable membrane, resulting in the loss of K+, water loss, and decreased deformability?

Accepted Answer

A) Overhydrated hereditary stomatocytosis 
B) Dehydrated hereditary stomatocytosis 
C) Hereditary elliptocytosis 
D) Hereditary spherocytosis 
A) Overhydrated hereditary stomatocytosis 
B) Dehydrated hereditary stomatocytosis 
C) Hereditary elliptocytosis 
D) Hereditary spherocytosis

Question 14

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

Accepted Answer

A) Potassium 
B) Chloride 
C) Sodium and water 
D) Sodium 
A) Potassium 
B) Chloride 
C) Sodium and water 
D) Sodium

Question 15

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

Accepted Answer

A) HS 
B) HE 
C) Acanthocytosis 
D) PNH 
A) HS 
B) HE 
C) Acanthocytosis 
D) PNH

Question 16

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

Accepted Answer

A) HPP 
B) HE 
C) HS 
D) Acanthocytosis 
A) HPP 
B) HE 
C) HS 
D) Acanthocytosis

Question 17

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 not corrected with glucose.What disorder is associated with these findings?

Accepted Answer

A) Hereditary pyropoikilocytosis 
B) Hereditary stomatocytosis 
C) PNH 
D) Hereditary spherocytosis 
A) Hereditary pyropoikilocytosis 
B) Hereditary stomatocytosis 
C) PNH 
D) Hereditary spherocytosis

Question 18

Hereditary pyropoikilocytosis differs in red cell morphology from other erythrocyte membrane defects by what characteristic cell shape?

Accepted Answer

A) Spherocytes 
B) Elliptocytes 
C) Schistocytes 
D) Target cells 
A) Spherocytes 
B) Elliptocytes 
C) Schistocytes 
D) Target cells

Question 19

A patient is suspected of having HS.However there appear to be less than 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?

Accepted Answer

A) Incubated osmotic fragility test 
B) Immunophenotyping for CD55 and CD59 
C) Sucrose hemolysis test 
D) Ham test 
A) Incubated osmotic fragility test 
B) Immunophenotyping for CD55 and CD59 
C) Sucrose hemolysis test 
D) Ham test

Question 20

List one cause of horizontal interactions of skeletal protein abnormalities?

Accepted Answer

A) Ankyrin 
B) Glycophorin C 
C) Band 3 
D) Protein 4.2 
A) Ankyrin 
B) Glycophorin C 
C) Band 3 
D) Protein 4.2

Exam 14: Hemolytic Anemia: Membrane Defects

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

The function of the MIRL is to:

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

Explain why patients with HS typically have an MCHC greater than 36%.

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

In PNH, cells are susceptible to complement induced lysis.This is thought to be due to the lack of what two regulating factors?

A defect in ankyrin is described as:

Hereditary stomatocytosis is a condition resulting from defects in:

PNH is currently detected in the lab by which of the following?

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

A sample has the following test results: hemoglobin 9 g/dL; autohemolysis is increased after 48 hours; increased hemolysis is observed with the Ham test and sucrose hemolysis test.Based on these findings, what is the patient most likely suffering from?

What disorder has an abnormal erythrocyte membrane that is abnormally permeable membrane, resulting in the loss of K+, water loss, and decreased deformability?

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

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

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

Hereditary pyropoikilocytosis differs in red cell morphology from other erythrocyte membrane defects by what characteristic cell shape?

A patient is suspected of having HS.However there appear to be less than 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?

List one cause of horizontal interactions of skeletal protein abnormalities?

Introduction

Cellular Homeostasis

Hematopoiesis

Structure and Function of Hematopoietic Organs

The Erythrocyte

Hemoglobin

The Leukocyte

Introduction to Anemia

Anemias of Disordered Iron Metabolism and Heme Synthesis

Hemoglobinopaties: Qualitative Defects

Thalassemia

Megaloblastic and Nonmegaloblastic Macrocytic Anemias

Introduction to Hemolytic Anemia

Hemolytic Anemia: Enzyme Deficiencies

Hemolytic Anemia: Immune Anemias

Hemolytic Anemia: Nonimmune Defects

Nonmalignant Granulocyte and Monocyte Disorders

Nonmalignant Lymphocyte Disorders

Introduction to Hematopoietic Neoplasms

Myeloproliferative Disorders

Myelodysplastic Syndromes

Acute Myeloid Leukemias

Acute Lymphoblastic Leukemias

Lymphoid Malignancies: Chronic Lymphoid Leukemias, Lymphomas, and Plasma Cell Neoplasms

Hematopoietic Stem Cell Transplantation

Morphologic Analysis of Body Fluids in the Hematology Laboratory

Primary Hemostasis

Secondary Hemostasis and Fibrinolysis

Disorders of Primary Hemostasis

Disorders of Secondary Hemostasis

Thrombophilia

Hematology Procedures and Hemostasis

Bone Marrow Examination

Automation in Hematology and Hemostasis

Flow Cytometry

Chromosome Analysis of Hematopoietic and Lymphoid Disorders

Molecular Analysis of Hematologic Diseases

Laboratory Testing in Coagulation

Quality Assessment in the Hematology Laboratory

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