Acquired Aplastic Anemia and PRCA Quiz

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Questions and Answers

Which technique is most recommended to provide reliable information about bone marrow cellularity?

Aspirate sampling

Fluid cytology

Bone marrow biopsy (correct)

Blood sample analysis

What is the primary characteristic of bone marrow in aplastic anemia?

Presence of regenerating lymphocytes

Presence of numerous progenitor cells

Hypocellularity and increased fat (correct)

Increased myeloid elements

What type of analysis is essential for assessing clonal cytogenetic abnormalities in bone marrow?

Bone marrow aspirate

Karyotype analysis (correct)

Complete blood count

Peripheral blood smear

Which statement about fluorescent in situ hybridization (FISH) studies is true?

FISH is specific to the chromosomal abnormality tested. Signup and view all the answers

Why should blood products be irradiated in patients with severe aplastic anemia?

To prevent allosensitization and infection risk Signup and view all the answers

What is a potential cause of pure red cell aplasia (PRCA)?

Exposure to hazardous drugs Signup and view all the answers

What might be seen in the bone marrow of young children with aplastic anemia?

Residual lymphocytes Signup and view all the answers

What critical information can bone marrow biopsies provide regarding the microenvironment?

Presence of fibrous or granulomatous changes Signup and view all the answers

What is a critical step in distinguishing between inherited and acquired causes of aplastic anemia?

A detailed medical history and laboratory evaluation Signup and view all the answers

What condition describes a reduction in the effective production of erythrocytes, granulocytes, and platelets by the bone marrow?

Aplastic anemia Signup and view all the answers

Which of the following is TRUE regarding the management of aplastic anemia?

Identification of the underlying cause is crucial before starting therapy. Signup and view all the answers

Which factor plays a significant role in the clinical evaluation of patients with aplastic anemia?

Bone marrow histological examination Signup and view all the answers

In the context of Pure Red Cell Aplasia (PRCA), which of the following can be considered a secondary cause?

Autoimmune disorders Signup and view all the answers

What is a common consequence of severe aplastic anemia that affects blood composition?

Pancytopenia Signup and view all the answers

Which of the following treatments is NOT typically considered for patients with aplastic anemia?

Increased iron supplementation Signup and view all the answers

What cellular component is notably affected in the pathophysiology of bone marrow failure syndromes like aplastic anemia?

Telomere shortening Signup and view all the answers

What is the significance of obtaining a detailed medication, environmental, and infectious history prior to diagnosis in aplastic anemia?

It aids in identifying potential causes of marrow suppression. Signup and view all the answers

Why is early HLA typing considered important in the management of aplastic anemia?

It guides future therapies by establishing donor compatibility. Signup and view all the answers

Which of the following is NOT typically a hemorrhagic manifestation from thrombocytopenia?

Bone pain Signup and view all the answers

What role does the complete blood count serve in the evaluation of aplastic anemia?

It defines the severity of cytopenias. Signup and view all the answers

How can telomere length be relevant to hematology, especially in conditions like dyskeratosis congenita?

Short telomeres can be associated with hematological malignancies. Signup and view all the answers

Which laboratory test is crucial for differentiating malignant processes from benign hematological conditions?

Bone marrow biopsy Signup and view all the answers

What type of chromosomal breakage analysis is associated with Fanconi anemia?

Diepoxybutane or mitomycin C testing Signup and view all the answers

What is the purpose of performing renal and hepatic laboratory evaluations before starting certain therapies?

To assess for potential toxicity of treatments. Signup and view all the answers

Which of the following is a potential cause of pure red cell aplasia (PRCA)?

Chronic infection Signup and view all the answers

What laboratory test is used to assess the presence of autoimmune cytopenias?

Coombs test Signup and view all the answers

What does the reticulocyte count help differentiate in patients with anemia?

Production versus destruction of red blood cells Signup and view all the answers

What evaluation is critical for assessing the severity of aplastic anemia?

Bone marrow aspirate Signup and view all the answers

Which of the following does NOT typically indicate a need for genetic testing in inherited marrow failure syndromes?

Presence of macrocytosis Signup and view all the answers

What is a common manifestation of congenital marrow failure syndromes?

Non-blanching petechiae Signup and view all the answers

Study Notes

Acquired Aplastic Anemia and Pure Red Cell Aplasia

Acquired Aplastic Anemia (AA) and Pure Red Cell Aplasia (PRCA) are conditions characterized by diminished production of mature blood cells by the bone marrow.
AA is a potentially fatal condition where the bone marrow is hypocellular, with empty spaces and an increase in fat cells, reticulum cells, plasma cells, and mast cells.
These conditions can be inherited or acquired. The chapter emphasizes the acquired form, as inherited cases are discussed in Chapter 7.
It is crucial to differentiate between acquired and inherited causes of aplastic anemia as the management and treatment options differ.
The severity of AA is categorized by Camitta's criteria, classifying patients as having severe AA when two or more of the following are present:
- Absolute neutrophil count (ANC) < 500/µL
- Platelet count < 20,000/µL
- Reticulocyte count < 1%
In addition to bone marrow aspiration, biopsies are essential for assessing bone marrow architecture, such as fibrosis or granulomas, to help differentiate AA from other conditions.
Other important investigations include:
- Karyotype and FISH analysis to detect clonal abnormalities associated with myelodysplasia or malignancy.
- Culture and DNA-based antigen detection to identify infectious agents.
- Complete red cell phenotyping to minimize the risk of erythrocyte antibodies in patients requiring long-term blood product support.
Blood products should be irradiated to reduce the risk of infection transmission and allosensitization, as patients with AA may require hematopoietic stem cell transplantation.
A detailed developmental history can help screen for potential congenital, metabolic, or storage diseases.
Early HLA typing of the patient and family members is beneficial for future treatment planning.
Baseline renal and hepatic function tests are recommended prior to initiating therapies that may potentially harm these organs.

Management and Outcome

Management focuses on identifying and eliminating potential exposures to hazardous drugs or toxins.
Patients requiring transfusions need to receive blood products from unrelated donors due to the risk of sensitization to family members' blood.
Blood products should be irradiated to reduce the risk of infection transmission and allosensitization.
Leukoreduction of blood products is prudent to reduce the risk of infection transmission and allosensitization.

Pure Red Cell Aplasia

PRCA is a specific form of marrow failure where there is a selective absence of erythropoiesis, resulting in anemia.
It is classified into primary acquired PRCA and secondary acquired PRCA.
Primary PRCA occurs without any identifiable underlying cause, while secondary PRCA is related to a specific precipitating factor.
Common causes of secondary PRCA include parvovirus B19 infection, autoimmune diseases, and medications.

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Description

This quiz explores Acquired Aplastic Anemia (AA) and Pure Red Cell Aplasia (PRCA), focusing on their characteristics, classifications, and management strategies. It emphasizes the importance of distinguishing between acquired and inherited forms, particularly regarding treatment options. Test your knowledge on these critical hematological conditions.