ICD-10: D60

Acquired pure red cell aplasia (APRA), classified under ICD-10 code D60, is a hematological condition characterized by a significant reduction in red blood cell production due to the failure of erythroid progenitor cells in the bone marrow. This condition can be associated with various underlying causes, including autoimmune disorders, infections, and certain medications. Below are alternative names and related terms for this condition:

Alternative Names

1. Erythroblastopenia: This term is often used interchangeably with acquired pure red cell aplasia, emphasizing the deficiency of erythroblasts in the bone marrow.
2. Acquired aplastic anemia: While this term generally refers to a broader category of bone marrow failure, it can sometimes encompass cases of pure red cell aplasia when the red cell lineage is specifically affected.
3. Hypoplastic anemia: This term may be used to describe the reduced production of red blood cells, although it is less specific than APRA.
4. Secondary pure red cell aplasia: This term highlights that the condition is acquired rather than congenital, indicating that it arises from external factors or diseases.

Related Terms

1. Bone marrow failure: A broader term that includes various conditions where the bone marrow does not produce sufficient blood cells, including red blood cells, white blood cells, and platelets.
2. Autoimmune hemolytic anemia: This condition can sometimes lead to secondary pure red cell aplasia, as the immune system mistakenly attacks red blood cells.
3. Parvovirus B19 infection: This viral infection is known to cause transient aplastic crises, particularly in individuals with underlying hemolytic anemia or other conditions affecting red blood cell production.
4. Thymoma-associated pure red cell aplasia: A specific association where pure red cell aplasia occurs in patients with thymoma, a tumor of the thymus gland.

Conclusion

Understanding the alternative names and related terms for acquired pure red cell aplasia is crucial for accurate diagnosis and treatment. These terms reflect the condition's complexity and its potential associations with other medical issues. If you have further questions or need more detailed information about this condition, feel free to ask!

Acquired pure red cell aplasia (PRCA), classified under ICD-10 code D60, is a hematological condition characterized by a significant reduction in red blood cell precursors in the bone marrow, leading to anemia. The diagnosis of PRCA involves several criteria and considerations, which can be categorized into clinical, laboratory, and histological evaluations.

Clinical Criteria

1. Symptoms of Anemia: Patients typically present with symptoms related to anemia, such as fatigue, pallor, shortness of breath, and tachycardia. The severity of symptoms often correlates with the degree of anemia.

2. Exclusion of Other Causes: It is crucial to rule out other forms of anemia, such as iron deficiency anemia, aplastic anemia, or anemia of chronic disease. This involves a thorough patient history and physical examination.

Laboratory Criteria

1. Complete Blood Count (CBC): A CBC will typically show:
   - Low hemoglobin levels indicating anemia.
   - Normal white blood cell and platelet counts, which helps differentiate PRCA from aplastic anemia.

2. Reticulocyte Count: A low reticulocyte count is a hallmark of PRCA, indicating inadequate production of new red blood cells by the bone marrow.

3. Bone Marrow Biopsy: A definitive diagnosis often requires a bone marrow biopsy, which typically reveals:
   - A marked reduction or absence of erythroid progenitor cells (the precursors to red blood cells).
   - Normal myeloid and megakaryocyte lineages, which helps confirm that the aplasia is specific to red blood cells.

4. Additional Tests: Depending on the suspected underlying cause, additional tests may include:
   - Direct Coombs test to check for autoimmune hemolytic anemia.
   - Viral serologies (e.g., parvovirus B19, hepatitis, HIV) to identify infectious causes of PRCA.
   - Autoantibody tests if an autoimmune etiology is suspected.

Histological Criteria

1. Bone Marrow Examination: The histological examination of the bone marrow is critical. It should show:
   - A significant reduction in erythroid cells.
   - Preserved myeloid and megakaryocyte lineages, confirming the diagnosis of PRCA rather than other forms of bone marrow failure.

Conclusion

The diagnosis of acquired pure red cell aplasia (ICD-10 code D60) is based on a combination of clinical presentation, laboratory findings, and histological examination of the bone marrow. It is essential to exclude other causes of anemia to confirm the diagnosis accurately. If you suspect PRCA, a comprehensive evaluation is necessary to guide appropriate management and treatment strategies.

Acquired pure red cell aplasia (PRCA), classified under ICD-10 code D60, is a hematological condition characterized by a significant reduction in red blood cell precursors in the bone marrow, leading to anemia. This condition can be caused by various factors, including autoimmune diseases, infections, and certain medications. The treatment approaches for acquired PRCA focus on addressing the underlying cause, managing symptoms, and restoring red blood cell production.

Treatment Approaches for Acquired Pure Red Cell Aplasia

1. Identifying and Treating Underlying Causes

• Autoimmune Disorders: If PRCA is secondary to an autoimmune condition, such as systemic lupus erythematosus (SLE), treatment may involve immunosuppressive therapies, including corticosteroids or other immunosuppressants to reduce the immune system's attack on red blood cell precursors[1].
• Infections: In cases where viral infections (e.g., parvovirus B19) are implicated, supportive care and monitoring may be sufficient, as the body often clears the infection on its own. In severe cases, intravenous immunoglobulin (IVIG) may be administered to help manage the infection and its effects on red blood cell production[2].
• Medications: If a specific medication is identified as the cause, discontinuation of the offending agent is crucial. In some cases, alternative medications may be considered[3].

2. Supportive Care

• Transfusions: Blood transfusions may be necessary to manage severe anemia and alleviate symptoms. This approach provides immediate relief but does not address the underlying issue of red blood cell production[4].
• Erythropoiesis-Stimulating Agents (ESAs): In certain cases, ESAs like erythropoietin may be used to stimulate the bone marrow to produce more red blood cells, particularly if the aplastic anemia is not severe and the bone marrow is still capable of responding[5].

3. Immunosuppressive Therapy

• For patients with severe PRCA, particularly those with an autoimmune component, immunosuppressive therapy may be indicated. This can include agents such as azathioprine, cyclosporine, or mycophenolate mofetil, which help to dampen the immune response and promote red blood cell production[6].

4. Bone Marrow Stimulation

• In some cases, treatments aimed at stimulating the bone marrow may be beneficial. This can include the use of granulocyte-colony stimulating factor (G-CSF) to enhance overall bone marrow function, although its effectiveness specifically for PRCA may vary[7].

5. Consideration of Stem Cell Transplantation

• In cases where PRCA is severe and unresponsive to other treatments, hematopoietic stem cell transplantation may be considered, particularly if there is an underlying genetic or severe aplastic component[8].

Conclusion

The management of acquired pure red cell aplasia requires a comprehensive approach that includes identifying and treating underlying causes, providing supportive care, and considering immunosuppressive therapies when appropriate. Each treatment plan should be tailored to the individual patient, taking into account the severity of the condition, the presence of any underlying diseases, and the patient's overall health status. Regular monitoring and follow-up are essential to assess treatment efficacy and make necessary adjustments.

For patients experiencing symptoms of anemia or those diagnosed with PRCA, it is crucial to work closely with a healthcare provider to determine the most effective treatment strategy tailored to their specific needs.

Acquired pure red cell aplasia (APRA), classified under ICD-10 code D60, is a hematological condition characterized by a significant reduction in red blood cell production due to the failure of erythroid progenitor cells in the bone marrow. This condition leads to anemia, which can manifest with various clinical symptoms and complications.

Clinical Description

Definition and Pathophysiology

Acquired pure red cell aplasia is defined as a selective failure of erythropoiesis, where the bone marrow produces an insufficient number of red blood cells while maintaining normal production of white blood cells and platelets. This condition can arise from various etiologies, including autoimmune disorders, infections, malignancies, and exposure to certain drugs or toxins. The underlying mechanism often involves immune-mediated destruction of erythroid progenitor cells or suppression of erythropoiesis.

Symptoms

Patients with APRA typically present with symptoms of anemia, which may include:
- Fatigue and weakness
- Pallor (pale skin)
- Shortness of breath, especially during exertion
- Dizziness or lightheadedness
- Tachycardia (increased heart rate)

In severe cases, patients may experience more serious complications, such as heart failure or angina due to inadequate oxygen delivery to tissues.

Diagnosis

The diagnosis of acquired pure red cell aplasia is primarily based on clinical evaluation and laboratory findings. Key diagnostic steps include:
- Complete Blood Count (CBC): This test typically shows a low hemoglobin level and a reduced reticulocyte count, indicating inadequate red blood cell production.
- Bone Marrow Biopsy: A bone marrow examination may reveal a marked reduction or absence of erythroid precursors while other hematopoietic lineages remain intact.
- Additional Tests: Depending on the suspected underlying cause, tests for autoimmune markers, viral infections (such as parvovirus B19), and other relevant investigations may be performed.

Treatment

Management of acquired pure red cell aplasia focuses on addressing the underlying cause and may include:
- Immunosuppressive Therapy: In cases where an autoimmune process is suspected, corticosteroids or other immunosuppressive agents may be used.
- Transfusions: Red blood cell transfusions can be administered to manage severe anemia and alleviate symptoms.
- Erythropoiesis-Stimulating Agents: In some cases, medications that stimulate red blood cell production may be beneficial.
- Treatment of Underlying Conditions: If APRA is secondary to an infection or malignancy, treating the primary condition is crucial.

Conclusion

Acquired pure red cell aplasia (ICD-10 code D60) is a serious hematological disorder that requires prompt diagnosis and management to prevent complications associated with anemia. Understanding the clinical presentation, diagnostic criteria, and treatment options is essential for healthcare providers to effectively care for patients with this condition. Early intervention can significantly improve patient outcomes and quality of life.

Acquired pure red cell aplasia (PRCA), classified under ICD-10 code D60, is a hematological condition characterized by a significant reduction in red blood cell production due to the absence or dysfunction of erythroid progenitor cells in the bone marrow. This condition can lead to severe anemia and is often associated with various underlying causes, including autoimmune disorders, infections, and certain medications.

Clinical Presentation

Signs and Symptoms

Patients with acquired pure red cell aplasia typically present with symptoms related to anemia, which may include:

• Fatigue and Weakness: Due to decreased oxygen-carrying capacity of the blood, patients often experience significant fatigue and general weakness.
• Pallor: A noticeable paleness of the skin and mucous membranes is common, resulting from reduced hemoglobin levels.
• Shortness of Breath: Patients may experience dyspnea, especially during physical exertion, as the body struggles to meet oxygen demands.
• Dizziness or Lightheadedness: These symptoms can occur due to reduced blood flow and oxygenation to the brain.
• Tachycardia: An increased heart rate may be observed as the body compensates for low oxygen levels.
• Headaches: Some patients report headaches, which can be attributed to anemia-related hypoxia.

Patient Characteristics

Acquired PRCA can affect individuals of any age, but certain characteristics may be more prevalent in specific populations:

• Age: While it can occur in children and adults, it is more commonly diagnosed in adults, particularly those in middle age.
• Gender: There may be a slight female predominance, especially in cases associated with autoimmune conditions.
• Underlying Conditions: Patients with autoimmune diseases (such as systemic lupus erythematosus or rheumatoid arthritis), viral infections (like parvovirus B19), or those undergoing certain treatments (such as chemotherapy) are at higher risk for developing PRCA.
• History of Infections: A history of viral infections, particularly those known to affect erythropoiesis, can be a significant factor in the development of acquired PRCA.

Diagnostic Evaluation

Laboratory Findings

Diagnosis of acquired PRCA typically involves a combination of clinical evaluation and laboratory tests:

• Complete Blood Count (CBC): This will show anemia with low hemoglobin and hematocrit levels, and a low reticulocyte count, indicating inadequate red blood cell production.
• Bone Marrow Biopsy: A key diagnostic tool, this procedure reveals a marked reduction or absence of erythroid precursors while other hematopoietic lineages remain intact.
• Serological Tests: Tests for viral infections (e.g., parvovirus B19, hepatitis, HIV) and autoimmune markers may be conducted to identify underlying causes.

Differential Diagnosis

It is essential to differentiate acquired PRCA from other forms of anemia, such as aplastic anemia or anemia of chronic disease, which may present with similar symptoms but have different underlying mechanisms and treatment approaches.

Conclusion

Acquired pure red cell aplasia is a serious condition that requires prompt recognition and management. Understanding the clinical presentation, signs, symptoms, and patient characteristics is crucial for healthcare providers to facilitate timely diagnosis and appropriate treatment. If you suspect PRCA in a patient, a thorough evaluation including history, physical examination, and targeted laboratory tests is essential to confirm the diagnosis and identify any underlying causes that may require specific interventions.