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Questions and Answers
What is the most common cause of neutropenia in adults who are ambulatory?
What is the most common cause of neutropenia in adults who are ambulatory?
Which condition does NOT directly reduce bone marrow reserve of neutrophils?
Which condition does NOT directly reduce bone marrow reserve of neutrophils?
Which factor is a rare cause of neutropenia?
Which factor is a rare cause of neutropenia?
How long do circulating neutrophils typically survive in the bloodstream?
How long do circulating neutrophils typically survive in the bloodstream?
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Which of the following is commonly associated with secondary autoimmune neutropenia?
Which of the following is commonly associated with secondary autoimmune neutropenia?
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What classification of neutropenia is indicated by a defect in granulopoiesis?
What classification of neutropenia is indicated by a defect in granulopoiesis?
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Which mechanism is involved in the genetic disorder known as Kostmann syndrome?
Which mechanism is involved in the genetic disorder known as Kostmann syndrome?
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Which type of neutropenia is characterized by the ability to accumulate normal bone marrow reserves?
Which type of neutropenia is characterized by the ability to accumulate normal bone marrow reserves?
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What is the inheritance pattern of Kostmann syndrome?
What is the inheritance pattern of Kostmann syndrome?
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What is a potential severe outcome of primary neutropenia as indicated in the content?
What is a potential severe outcome of primary neutropenia as indicated in the content?
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What is the normal range for absolute neutrophil count (ANC) in adults?
What is the normal range for absolute neutrophil count (ANC) in adults?
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Which of the following is NOT a primary cause of neutropenia?
Which of the following is NOT a primary cause of neutropenia?
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In what age group is infection cited as the most common cause of neutropenia?
In what age group is infection cited as the most common cause of neutropenia?
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What is the lower limit of ANC for infants according to laboratory definitions?
What is the lower limit of ANC for infants according to laboratory definitions?
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Which group has an ANC range that can occasionally be as low as 1.0 to 1.5×10^9/L?
Which group has an ANC range that can occasionally be as low as 1.0 to 1.5×10^9/L?
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Which of the following is a constitutional disorder associated with neutropenia?
Which of the following is a constitutional disorder associated with neutropenia?
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Which laboratory specialty contributes to the diagnosis of neutropenia?
Which laboratory specialty contributes to the diagnosis of neutropenia?
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How is neutropenia classified in adults?
How is neutropenia classified in adults?
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What is a common viral infection that is associated with immune-mediated neutropenia?
What is a common viral infection that is associated with immune-mediated neutropenia?
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Which condition is characterized by a maturation arrest in the myeloid lineage?
Which condition is characterized by a maturation arrest in the myeloid lineage?
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Which of the following congenital conditions is NOT commonly associated with mild neutropenia?
Which of the following congenital conditions is NOT commonly associated with mild neutropenia?
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Which factor is important in the clinical history of a neonate potentially experiencing neutropenia?
Which factor is important in the clinical history of a neonate potentially experiencing neutropenia?
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What is the possible inheritance mechanism of chronic benign neutropenia?
What is the possible inheritance mechanism of chronic benign neutropenia?
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What is the diagnostic frequency of chronic benign neutropenia before bone marrow examination?
What is the diagnostic frequency of chronic benign neutropenia before bone marrow examination?
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What is one of the rare causes of neutropenia that involves tetraploid nuclei?
What is one of the rare causes of neutropenia that involves tetraploid nuclei?
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Which of the following statements accurately describes the nature of neonatal neutropenia related to maternal conditions?
Which of the following statements accurately describes the nature of neonatal neutropenia related to maternal conditions?
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What mutation is associated with Shwachman-Diamond syndrome?
What mutation is associated with Shwachman-Diamond syndrome?
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What is a common clinical feature of cyclic neutropenia?
What is a common clinical feature of cyclic neutropenia?
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What clinical characteristic is associated with Chediak-Higashi syndrome?
What clinical characteristic is associated with Chediak-Higashi syndrome?
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What percentage of patients with secondary lymphocytic leukemia show increased apoptosis?
What percentage of patients with secondary lymphocytic leukemia show increased apoptosis?
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What common factor is noted in drug-induced suppression of myelopoiesis?
What common factor is noted in drug-induced suppression of myelopoiesis?
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What is the inheritance pattern of Shwachman-Diamond syndrome?
What is the inheritance pattern of Shwachman-Diamond syndrome?
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Which factor significantly impacts the severity of neutropenia in drug-induced cases?
Which factor significantly impacts the severity of neutropenia in drug-induced cases?
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What laboratory finding can indicate ineffective nutritional marrow suppression?
What laboratory finding can indicate ineffective nutritional marrow suppression?
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Which disorder is characterized by giant granules in many cells?
Which disorder is characterized by giant granules in many cells?
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What is a common diagnostic approach for severe congenital neutropenia?
What is a common diagnostic approach for severe congenital neutropenia?
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Flashcards
Neutropenia
Neutropenia
A condition characterized by an abnormally low neutrophil count.
Normal ANC range
Normal ANC range
The absolute neutrophil count (ANC) for adults ranges from 1.5 to 7.0×10^9/L.
Neutropenia classification
Neutropenia classification
Neutropenia is classified based on absolute neutrophil count into three groups.
Neutropenia in neonates
Neutropenia in neonates
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Primary causes of neutropenia
Primary causes of neutropenia
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Secondary causes of neutropenia
Secondary causes of neutropenia
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Age-related causes of neutropenia
Age-related causes of neutropenia
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Importance of ethnicity
Importance of ethnicity
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Neutropenia causes
Neutropenia causes
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Neutrophil lifespan
Neutrophil lifespan
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Bone marrow reserves
Bone marrow reserves
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Myeloablative therapies
Myeloablative therapies
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Autoimmune neutropenia
Autoimmune neutropenia
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Decreased BM Reserve
Decreased BM Reserve
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Primary Neutropenia
Primary Neutropenia
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Secondary Neutropenia
Secondary Neutropenia
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Kostmann Syndrome
Kostmann Syndrome
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Causes of Neutropenia
Causes of Neutropenia
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Viral Infections and Neutropenia
Viral Infections and Neutropenia
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Clinical History in Neutropenia
Clinical History in Neutropenia
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Severe Neutropenia Risks
Severe Neutropenia Risks
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Congenital Neutropenia Conditions
Congenital Neutropenia Conditions
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Importance of Antenatal Care
Importance of Antenatal Care
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Diagnosis of Neutropenia
Diagnosis of Neutropenia
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Cyclic Neutropenia
Cyclic Neutropenia
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Chediak-Higashi Syndrome
Chediak-Higashi Syndrome
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Exocrine Function
Exocrine Function
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Neutropenia in Chemotherapy
Neutropenia in Chemotherapy
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Drug-Induced Neutropenia
Drug-Induced Neutropenia
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Granulocyte Morphology
Granulocyte Morphology
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Congenital Neutropenia
Congenital Neutropenia
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Recurrent Fever
Recurrent Fever
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FAS Ligand
FAS Ligand
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Protein-Calorie Malnutrition
Protein-Calorie Malnutrition
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Study Notes
Neutropenia Overview
- Neutropenia is a common laboratory finding in adults and children
- Underlying causes are heterogeneous, including benign conditions, autoimmune disorders, infections, and malignancies
- Clinical labs are crucial for diagnosis, using hematology, microbiology, molecular biology/cytogenetics, and clinical chemistry
Defining Neutropenia
- Normal adult absolute neutrophil count (ANC) ranges from 1.5 to 7.0 x 109/L
- Lower limit of ANC varies by patient age and race
- Neonates/infants lower limit is approximately 2.5 x 109/L
- Up to 25% of African-American children/adults have ANC ranging from 1.0 to 1.5 x 109/L
How Neutropenia is classified
- Most labs classify neutropenia in infants with ANC < 2.5 x 109/L and in adults with ANC < 1.5 x 109/L
- Stratified into mild, moderate and severe based on ANC values and clinical context
- Mild: ANC 1.0-1.5 x 109/L, general good health, usually no infection
- Moderate: ANC 0.5-1.0 x 109/L, associated disease, debilitated/malnourished, usually minimal to moderate infection risk
- Severe: ANC < 0.5 x 109/L, all clinical settings, moderate to severe infection risk
Age-Related Causes of Neutropenia
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Neonates: Infection (most common), maternal hypertension/drug treatment, maternal antibody production, constitutional disorders (e.g., cyclic neutropenia, Kostmann syndrome, Chediak-Higashi syndrome)
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Infants/Children: Infections, autoimmune neutropenia, neoplasms replacing bone marrow, idiosyncratic drug reactions, secondary autoimmune neutropenia in collagen vascular disorders, immunodeficiency disorders, megaloblastic anemia, myeloablative therapies, constitutional neutropenia disorders (rare), copper deficiency (rare)
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Adults: Idiosyncratic drug reactions (most common in outpatients), infections, neoplasms replacing bone marrow, myeloablative therapies, secondary autoimmune neutropenia in collagen vascular disorders, autoimmune disorders (including white blood cell aplasia)
Classification of Neutropenia by Marrow Reserve
- Neutropenia with decreased bone marrow reserve (BM)
- Primary: defect in granulopoiesis.
- Secondary: suppression of normal granulopoiesis.
- Neutropenia with normal bone marrow reserve Different disorders responsible for severe congenital neutropenia have distinctive symptoms e.g., Schwachman-Diamond syndrome characterised by steatorrhea, short stature, and exocrine pancreatic deficiency
Classification of Neutropenias by Marrow Reserve and Clinical Features
- Table showing primary disorders, clinical traits, and diagnostic tools, distinguishing syndromes through characteristics and diagnostic tests
Cyclic Neutropenia, Chediak-Higashi Syndrome
- Cyclic neutropenia: Periodic apoptosis of precursors, often associated with ELA2 mutation; recurrent fever, skin/otolaryngeal infections, and no increased risk of hematological malignancies
- Chediak-Higashi syndrome: Mutation in the LYST gene, recurrent pyogenic infections, oculocutaneous albinism, decreased microbicidal activity (giant granules in cells)
Secondary Neutropenias
- Large granular lymphocytic leukaemia and related disorders
- Chemotherapy
- Drug-induced (non-immune)
- Nutritional deficiencies (protein-calorie malnutrition, folate/copper/B12 deficiency)
- Infections (viral like EBV,CMV, HIV, Hepatitis)
Neutropenia with Normal Bone Marrow Reserve
- Chronic/ benign neutropenia of infancy/childhood: Caused by antineutrophil antibodies
- Non immune chronic benign neutropenia: High incidence, increased apoptosis.
- Ethnic or Benign familial neutropenia: Unknown cause (unknown mechanism), incidence AD, findings mostly in family members (similar findings).
Alloimmune Neutropenia
- Maternal alloimmunization to HNA antigens (1a/1b/2a/1c/3a/4a)
- Moderate/severe neutropenia in newborns, cutaneous infections, UTIs, omphalitis
Drug-Induced Neutropenia
- Antibody or complement-mediated suppression of myelopoiesis
- Fever, sepsis, pneumonia, up to 50% mortality, 80% recovery rate
Infection-Related Neutropenia, Hypersplenism
- Virus-mediated antibody
- Sequestration, destruction
Diagnostic Approach to Neutropenia
- Detailed clinical history and physical examination first.
- Radiographic findings can be important
- Blood counts, Peripheral blood smear, Immunological testing, infectious disease testing, bone marrow biopsy
- Different approaches are used for children vs adults
Work-up for Pediatric and Adult Patients
- Flowcharts for evaluation and diagnostic approach, using clinical findings and lab values to guide further investigations (e.g. genetic syndromes, bone marrow biopsy)
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Test your knowledge on neutropenia in adults with this quiz. Explore questions about causes, classification, and specific conditions linked to neutropenia. Perfect for those studying hematology and clinical practices.