Leukocyte Disorders - Chapter 26 Quiz

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Questions and Answers

Which condition is associated with severe combined immune deficiency?

Bruton Tyrosine Kinase Deficiency (correct)

Wiskott-Aldrich Syndrome

Chédiak-Higashi Syndrome

Pelger-Huët Anomaly

What anomaly is characterized by neutrophil hypersegmentation?

Alder-Reilly Anomaly

Pseudo-Pelger-Huët Anomaly

May-Hegglin Anomaly

Neutrophil Hypersegmentation (correct)

Which syndrome is linked with leukocyte adhesion disorders?

Chronic Granulomatous Disease

WHIM Syndrome (correct)

22q11 Syndromes

Wiskott-Aldrich Syndrome

What is one of the defects associated with respiratory burst?

Chronic Granulomatous Disease Signup and view all the answers

Which of the following is NOT a morphologic abnormality of leukocytes?

Lysosomal Storage Diseases Signup and view all the answers

What are Velasco inclusions primarily composed of?

Ribosomal ribonucleic acid (RNA) Signup and view all the answers

Which condition is NOT typically associated with the presence of Döhle-like bodies?

Acute alcoholism Signup and view all the answers

What does the presence of pyknotic nuclei indicate?

Cell death Signup and view all the answers

What is a common finding associated with Ehrlichiosis and Anaplasmosis?

Thrombocytopenia Signup and view all the answers

Which statement regarding cytoplasmic vacuolation is correct?

It reflects phagocytosis. Signup and view all the answers

What feature differentiates hypogranular eosinophils from normal eosinophils?

Decreased granularity Signup and view all the answers

What characteristic is observed in necrotic nuclei?

Rounded nuclear fragments Signup and view all the answers

Basophil granules have which of the following properties?

They may wash away during staining. Signup and view all the answers

What term is recommended by the International Council for Standardization in Hematology (ICSH) when lymphocyte morphology suggests a malignant or clonal etiology?

Abnormal Signup and view all the answers

Which type of lymphocyte exhibits features characteristic of plasma cells?

Plasmacytoid lymphocytes Signup and view all the answers

Which virus is primarily responsible for causing Infectious Mononucleosis?

Epstein-Barr Virus Signup and view all the answers

What is a common laboratory finding in cases of Infectious Mononucleosis?

Absolute lymphocytosis with over 50% reactive forms Signup and view all the answers

Which of the following is NOT a common clinical manifestation of Infectious Mononucleosis?

Jaundice Signup and view all the answers

What is the cause of Leukocyte Adhesion Disorder I (LAD I)?

Mutation in ITGB2 Signup and view all the answers

Which aspect of LAD II is normal compared to LAD I?

Expression of integrins Signup and view all the answers

What is the primary defect in Chronic Granulomatous Disease (CGD)?

Inability to undergo respiratory burst Signup and view all the answers

Which infections are patients with Chronic Granulomatous Disease (CGD) primarily susceptible to?

Catalase positive bacterial infections Signup and view all the answers

What is a consequence of the inability of neutrophils and monocytes to move to the site of inflammation in LAD?

Recurrent infections, particularly mucosal and skin infections Signup and view all the answers

What does the abnormal selectin ligands synthesis in LAD II affect?

Leukocyte rolling Signup and view all the answers

What genetic element is affected in patients with LAD III?

Kindlin-3 gene Signup and view all the answers

What is a common clinical manifestation of both LAD I and LAD III?

Neutrophilia Signup and view all the answers

What is the primary genetic mutation associated with WHIM syndrome?

CXCR4 gene mutation Signup and view all the answers

What is one of the main clinical features of Pelger-Huët anomaly?

Decreased nuclear segmentation Signup and view all the answers

Which infection are patients with WHIM syndrome particularly susceptible to?

HPV infection Signup and view all the answers

What morphological characteristic is associated with true Pelger-Huët anomaly?

Round or peanut-shaped nuclei Signup and view all the answers

Which of the following conditions is associated with pseudo-Pelger-Huët anomaly?

Myelodysplastic syndrome (MDS) Signup and view all the answers

What is the typical number of lobes present in normal neutrophils?

3 to 5 lobes Signup and view all the answers

What type of anemia is neutrophil hypersegmentation primarily associated with?

Megaloblastic anemia Signup and view all the answers

Which abnormality results in the accumulation of neutrophils in the bone marrow?

Myelokathexis Signup and view all the answers

Study Notes

Leukocyte Disorders Overview

Congenital defects in leukocyte function and quantity can lead to severe immune deficiencies and various syndromes.
Key congenital defects include Severe Combined Immune Deficiency, Wiskott-Aldrich Syndrome, and Chédiak-Higashi Syndrome.

Morphologic Abnormalities of Leukocytes

Pelger-Huët Anomaly: An autosomal disorder characterized by decreased nuclear segmentation, caused by mutations in the Lamin β-receptor gene.
Pseudo-Pelger-Huët Anomaly: Associated with myelodysplastic syndromes and can be drug-induced; segmented neutrophils display similar morphology.
Neutrophil Hypersegmentation: Normal neutrophils have 3-5 lobes; hypersegmentation is associated with megaloblastic anemia.

Key Cytoplasmic Changes

Döhle-like bodies: Cytoplasmic inclusions indicative of conditions like bacterial infections and sepsis; can also occur in May-Hegglin anomaly.
Cytoplasmic Vacuolation: Reflects phagocytosis and can be caused by various factors including bacterial presence and radiation exposure.

Infectious Conditions

Ehrlichiosis and Anaplasmosis: Caused by bacteria transmitted via ticks, leading to leukopenia, thrombocytopenia, and high liver enzymes; diagnosed with PCR.
Infectious Mononucleosis: Caused by Epstein-Barr virus; presents with absolute lymphocytosis and increased reactive T cells.

Leukocyte Adhesion Disorders (LAD)

Results in impaired migration of neutrophils and monocytes to inflammation sites.
LAD I: Caused by a mutation in ITGB2 affecting β2 integrin; leads to infant mortality and recurrent infections.
LAD II and III: Involves fucose transporter and Kindlin-3 mutations, leading to integrin dysfunction and severe immunodeficiency.

Defects in Respiratory Burst

Chronic Granulomatous Disease (CGD): Inability of neutrophils to perform respiratory burst, causing susceptibility to catalase-positive infections; diagnosed using a fluorescent probe test.

WHIM Syndrome

Includes warts, hypogammaglobulinemia, infections, and myelokathexis resulting from CXCR4 gene mutations; leads to neutrophil accumulation in the bone marrow.

Lymphocyte Characteristics

Reactive lymphocytes, such as plasmacytoid and atypical forms, are associated with benign or malignant etiologies, respectively.
Plasmacytoid Lymphocytes: Increased in conditions like lymphoma.

Abnormal Nuclei

Pyknotic Nuclei: Indicate cell death; necrotic nuclei show rounded fragments without normal chromatin patterns.

Eosinophils and Basophils

Hypogranular Eosinophils: Linked to acute lymphoblastic leukemia; should be counted accurately in differential assessments.
Basophil Granules: Water-soluble; may wash away during staining.

Summary of Laboratory Findings

Testing for leukocyte disorders focuses on morphological changes, presence of inclusions, and response to infections. Key diagnostic criteria include the assessment of cellular morphology and counts in blood analyses.

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Description

Test your knowledge on leukocyte disorders with this quiz based on Chapter 26 of Rodak's 6th Edition. Explore congenital defects, morphologic abnormalities, and other important topics related to leukocytes. Perfect for students preparing for medical laboratory exams.