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Questions and Answers
Which condition is associated with severe combined immune deficiency?
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?
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?
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?
What is one of the defects associated with respiratory burst?
Which of the following is NOT a morphologic abnormality of leukocytes?
Which of the following is NOT a morphologic abnormality of leukocytes?
What are Velasco inclusions primarily composed of?
What are Velasco inclusions primarily composed of?
Which condition is NOT typically associated with the presence of Döhle-like bodies?
Which condition is NOT typically associated with the presence of Döhle-like bodies?
What does the presence of pyknotic nuclei indicate?
What does the presence of pyknotic nuclei indicate?
What is a common finding associated with Ehrlichiosis and Anaplasmosis?
What is a common finding associated with Ehrlichiosis and Anaplasmosis?
Which statement regarding cytoplasmic vacuolation is correct?
Which statement regarding cytoplasmic vacuolation is correct?
What feature differentiates hypogranular eosinophils from normal eosinophils?
What feature differentiates hypogranular eosinophils from normal eosinophils?
What characteristic is observed in necrotic nuclei?
What characteristic is observed in necrotic nuclei?
Basophil granules have which of the following properties?
Basophil granules have which of the following properties?
What term is recommended by the International Council for Standardization in Hematology (ICSH) when lymphocyte morphology suggests a malignant or clonal etiology?
What term is recommended by the International Council for Standardization in Hematology (ICSH) when lymphocyte morphology suggests a malignant or clonal etiology?
Which type of lymphocyte exhibits features characteristic of plasma cells?
Which type of lymphocyte exhibits features characteristic of plasma cells?
Which virus is primarily responsible for causing Infectious Mononucleosis?
Which virus is primarily responsible for causing Infectious Mononucleosis?
What is a common laboratory finding in cases of Infectious Mononucleosis?
What is a common laboratory finding in cases of Infectious Mononucleosis?
Which of the following is NOT a common clinical manifestation of Infectious Mononucleosis?
Which of the following is NOT a common clinical manifestation of Infectious Mononucleosis?
What is the cause of Leukocyte Adhesion Disorder I (LAD I)?
What is the cause of Leukocyte Adhesion Disorder I (LAD I)?
Which aspect of LAD II is normal compared to LAD I?
Which aspect of LAD II is normal compared to LAD I?
What is the primary defect in Chronic Granulomatous Disease (CGD)?
What is the primary defect in Chronic Granulomatous Disease (CGD)?
Which infections are patients with Chronic Granulomatous Disease (CGD) primarily susceptible to?
Which infections are patients with Chronic Granulomatous Disease (CGD) primarily susceptible to?
What is a consequence of the inability of neutrophils and monocytes to move to the site of inflammation in LAD?
What is a consequence of the inability of neutrophils and monocytes to move to the site of inflammation in LAD?
What does the abnormal selectin ligands synthesis in LAD II affect?
What does the abnormal selectin ligands synthesis in LAD II affect?
What genetic element is affected in patients with LAD III?
What genetic element is affected in patients with LAD III?
What is a common clinical manifestation of both LAD I and LAD III?
What is a common clinical manifestation of both LAD I and LAD III?
What is the primary genetic mutation associated with WHIM syndrome?
What is the primary genetic mutation associated with WHIM syndrome?
What is one of the main clinical features of Pelger-Huët anomaly?
What is one of the main clinical features of Pelger-Huët anomaly?
Which infection are patients with WHIM syndrome particularly susceptible to?
Which infection are patients with WHIM syndrome particularly susceptible to?
What morphological characteristic is associated with true Pelger-Huët anomaly?
What morphological characteristic is associated with true Pelger-Huët anomaly?
Which of the following conditions is associated with pseudo-Pelger-Huët anomaly?
Which of the following conditions is associated with pseudo-Pelger-Huët anomaly?
What is the typical number of lobes present in normal neutrophils?
What is the typical number of lobes present in normal neutrophils?
What type of anemia is neutrophil hypersegmentation primarily associated with?
What type of anemia is neutrophil hypersegmentation primarily associated with?
Which abnormality results in the accumulation of neutrophils in the bone marrow?
Which abnormality results in the accumulation of neutrophils in the bone marrow?
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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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