Non-Malignant Leukocyte Disorders

Multiple Choice

Primary immunodeficiency disorders are also known as:

• Acquired immune disorders

• Inborn errors of immunity

• Autoimmune diseases

• Malignant leukocyte disorders

Multiple Choice

Which of the following is NOT a characteristic of Severe Combined Immune Deficiency (SCID)?

• Marked decrease in circulating T cells

• Poorly functioning B cells

• Profound clinical manifestations in infancy

• Increased immunoglobulin levels

Multiple Select

Which of the following cell types are decreased in both Common Gamma Chain Deficiency and ADA Deficiency?

• T cells

• B cells

• NK cells

• Platelets

Fill in the Blank

Wiskott-Aldrich Syndrome is associated with decreased ___ protein, which is important in cytoskeletal remodeling and nuclear transcription in hematopoietic cells.

Multiple Choice

Which of the following syndromes is classified as a combined immunodeficiency that involves a microdeletion on chromosome 22?

• Wiskott-Aldrich Syndrome

• 22q11.2 Deletion Syndrome

• ADA Deficiency

• Common Gamma Chain Deficiency

Dropdown

Bruton Tyrosine Kinase (BTK) deficiency is also known as ​ ​

Select answer

Multiple Choice

Which of the following cell types are affected by the presence of fused lysosomal granules in Chediak Higashi Syndrome?

• Granulocytes

• Monocytes

• Large granular lymphocytes

• All of the above

Multiple Choice

Which of the following genes is affected in Chediak Higashi Syndrome?

• WAS

• IL2RG

• CHS1 LYST

• ITGB2

Fill in the Blank

Give one condition where Pseudo-Chediak Higashi granules are observed.

Multiple Choice

Which of the following features is associated with G6PC3 deficiency in severe congenital neutropenias?

• Neurologic defects

• BM dysplasia, anemia, deafness

• Short stature, hypothyroidism

• No response to G-CSF

Multiple Choice

Which gene is associated with Kostmann disease in severe congenital neutropenias?

• ELANE

• GFI1

• HAX1

• G6PC3

Multiple Choice

What is the primary functional consequence of leukocyte adhesion disorders (LAD)?

• Impaired phagocytosis

• Inability of neutrophils and monocytes to move from circulation to the site of infection

• Decreased antibody production

• Increased platelet aggregation

Fill in the Blank

Fill in the blank: LAD I is caused by a mutation in the ___ gene.

Multiple Select

Select all correct statements about LAD III.

• Mutation in Kindlin-3 is involved

• Leukocytes have abnormal expression of integrins

• Failure in responding to external signals leads to impaired leukocyte activation

• Decreased platelet GPIIb B3 results in bleeding

Multiple Choice

Which of the following is a characteristic of Job’s Syndrome?

• Directional motility of phagocytes is impaired

• Bone marrow reserves of granulocytes are poor

• Neutropenia is a consistent finding

• Phagocytes have defective bactericidal activity

Multiple Select

Which of the following are true about Lazy Leukocyte Syndrome?

• Both random and directed movement are defective

• Bone marrow reserves of granulocytes are normal

• Neutropenia is a consistent finding

• Cells have increased phagocytic activity

Multiple Choice

Chronic Granulomatous Disease is most commonly inherited in which manner?

• X-linked recessive

• Autosomal dominant

• Autosomal recessive

• Mitochondrial

Open Ended

Explain how the Nitroblue Tetrazolium (NBT) Reduction Test helps in diagnosing Chronic Granulomatous Disease.

Fill in the Blank

The gene CYBB, associated with Chronic Granulomatous Disease, is located on chromosome ___

.

Multiple Choice

Which of the following best describes the function of Myeloperoxidase (MPO) in phagocytes?

• Catalyzes the conversion of hydrogen peroxide to hypochlorous acid

• Breaks down bacterial cell walls

• Promotes neutrophil chemotaxis

• Enhances antibody production

Multiple Choice

In Gaucher disease, which cytoplasmic appearance is classic for Gaucher cells in marrow?

• Clear vacuoles with bubbles

• Fibrillar, “wrinkled tissue‑paper” cytoplasm

• Dense azurophilic granules only

• Basophilic Döhle‑like inclusions

Multiple Choice

Niemann‑Pick cells most often show which feature on microscopic review?

• Honeycomb nuclei

• Bubble‑like cytoplasmic lipid deposits with eccentric nucleus

• Needle‑like Auer rods

• Iron granules within mitochondria

Multiple Choice

Congenital Pelger‑Huët anomaly (PHA) is caused by mutation in which gene?

• BTK

• LBR

• ADA

• WAS

Multiple Choice

Pseudo‑ or acquired Pelger‑Huët morphology is classically associated with:

• Thalassemia trait

• Iron deficiency anemia

• Myelodysplastic syndrome (MDS) and AML

• Vitamin B12 deficiency

Multiple Choice

Neutrophil hypersegmentation (>5 lobes) is most often linked to:

• Megaloblastic anemia

• Iron deficiency

• Acute infection

• Hemoglobinopathy

Multiple Choice

A hallmark of May‑Hegglin anomaly is the combination of:

• Pince‑nez nuclei

• Hypersegmented neutrophils only

• Hypogranular basophils

• Giant platelets with Döhle‑like inclusions

Multiple Choice

The most frequent cause of acquired neutropenia overall is:

• Viral infection

• Drug‑induced marrow suppression or immune reaction

• Autoimmune disease

• Congenital syndromes

Multiple Choice

A common pair of reactive changes in neutrophils during infection/inflammation is:

• Toxic granulation and Döhle bodies

• Giant platelets and basophilia

• A common pair of reactive changes in neutrophils during infection/inflammation is:

• Alder‑Reilly granules and Auer rods

Multiple Choice

Basophil and eosinophil hypogranularity on stained films is often due to:

• Inherited granule disorders only

• Water‑soluble granules and staining washout

• Iron deficiency

• Folate deficiency

Multiple Choice

The typical hematologic pattern in infectious mononucleosis is:

• Neutrophilia with left shift only

• Lymphocytosis with atypical/reactive lymphocytes

• Basophilia with giant granules

• Pancytopenia