ICD-10: D60.9

Acquired pure red cell aplasia, unspecified

Additional Information

Description

Acquired pure red cell aplasia (PRCA) is a rare hematological condition characterized by a significant reduction in red blood cell production due to the failure of erythroid progenitor cells in the bone marrow. The ICD-10 code D60.9 specifically refers to "Acquired pure red cell aplasia, unspecified," indicating that the exact cause of the condition is not specified.

Clinical Description

Definition

Acquired PRCA is defined as a condition where the bone marrow fails to produce red blood cells, leading to anemia. This condition can occur due to various underlying factors, including autoimmune diseases, infections, certain medications, and malignancies. Unlike congenital forms of aplastic anemia, acquired PRCA typically arises later in life.

Symptoms

Patients with acquired PRCA may present with symptoms of anemia, which can include:
- Fatigue and weakness
- Pallor (pale skin)
- Shortness of breath, especially during exertion
- Dizziness or lightheadedness
- Increased heart rate (tachycardia)

Diagnosis

Diagnosis of acquired PRCA involves a combination of clinical evaluation, laboratory tests, and bone marrow examination. 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 biopsy may reveal a marked reduction or absence of erythroid precursors while other hematopoietic lineages remain intact.
- Additional Tests: Tests for underlying causes, such as viral infections (e.g., parvovirus B19), autoimmune disorders (e.g., systemic lupus erythematosus), or malignancies, may also be conducted.

Treatment

The management of acquired PRCA focuses on treating 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 may be necessary to manage severe anemia.
- Erythropoiesis-Stimulating Agents: Medications that stimulate red blood cell production may be considered, particularly in cases related to chronic kidney disease.
- Treatment of Underlying Conditions: Addressing any infections or malignancies contributing to the condition is crucial.

Conclusion

ICD-10 code D60.9 captures the essence of acquired pure red cell aplasia when the specific etiology is not identified. Understanding the clinical presentation, diagnostic approach, and treatment options is essential for healthcare providers managing patients with this condition. Early recognition and appropriate management can significantly improve patient outcomes and quality of life.

Clinical Information

Acquired pure red cell aplasia (PRCA), classified under ICD-10 code D60.9, 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 arise from various underlying causes, including autoimmune disorders, infections, and certain medications. Understanding the clinical presentation, signs, symptoms, and patient characteristics associated with this condition is crucial for effective diagnosis and management.

Clinical Presentation

Signs and Symptoms

Patients with acquired pure red cell aplasia typically present with symptoms related to anemia, which can vary in severity depending on the degree of red blood cell deficiency. Common signs and symptoms include:

  • Fatigue and Weakness: Due to decreased oxygen-carrying capacity of the blood, patients often experience significant fatigue and general weakness[1].
  • Pallor: A noticeable paleness of the skin and mucous membranes is common, resulting from reduced hemoglobin levels[1].
  • Shortness of Breath: Patients may experience dyspnea, especially during physical exertion, as the body struggles to meet oxygen demands[1].
  • Dizziness or Lightheadedness: This can occur due to low blood pressure or reduced cerebral perfusion from anemia[1].
  • Tachycardia: Increased heart rate may be observed as the body compensates for low oxygen levels[1].

Additional Symptoms

In some cases, patients may also report:

  • Headaches: Resulting from reduced oxygen delivery to the brain[1].
  • Cold Extremities: Due to poor circulation associated with anemia[1].
  • Chest Pain: In severe cases, patients may experience angina-like symptoms due to inadequate oxygenation of the heart muscle[1].

Patient Characteristics

Demographics

Acquired PRCA can affect individuals of any age, but certain demographic factors may influence its prevalence:

  • Age: It can occur in both children and adults, but the underlying causes may differ. For instance, in children, infections like parvovirus B19 are more common, while in adults, autoimmune diseases or malignancies may be more prevalent[1][2].
  • Gender: Some studies suggest a slight female predominance, particularly in cases associated with autoimmune disorders[2].

Risk Factors

Several risk factors may predispose individuals to develop acquired PRCA:

  • Autoimmune Disorders: Conditions such as systemic lupus erythematosus (SLE) or rheumatoid arthritis can lead to the development of PRCA due to the production of autoantibodies against erythroid progenitor cells[2].
  • Infections: Viral infections, particularly parvovirus B19, can cause transient aplastic crises, leading to PRCA[2].
  • Medications: Certain drugs, including some antibiotics and antiepileptics, have been implicated in the development of PRCA through immune-mediated mechanisms[2].
  • Malignancies: Hematological malignancies, such as lymphoproliferative disorders, can also be associated with PRCA due to the infiltration of the bone marrow or immune dysregulation[2].

Conclusion

Acquired pure red cell aplasia (ICD-10 code D60.9) presents primarily with symptoms of anemia, including fatigue, pallor, and shortness of breath. The condition can affect individuals across various demographics, with specific risk factors such as autoimmune diseases, infections, and certain medications playing a significant role in its development. Early recognition and management of the underlying causes are essential for improving patient outcomes and alleviating symptoms associated with this hematological disorder.

Approximate Synonyms

Acquired pure red cell aplasia (APRCA) is a hematological condition characterized by a significant reduction in red blood cell production, leading to anemia. The ICD-10 code for this condition is D60.9, which specifically denotes "Acquired pure red cell aplasia, unspecified." Below are alternative names and related terms associated with this condition.

Alternative Names for Acquired Pure Red Cell Aplasia

  1. Erythroblastopenia: This term is often used interchangeably with acquired pure red cell aplasia, emphasizing the reduction of erythroblasts (precursors to red blood cells) in the bone marrow[4].

  2. Aplastic Anemia: While this term broadly refers to a condition where the bone marrow fails to produce adequate amounts of blood cells, it can sometimes encompass cases of pure red cell aplasia, particularly when red cell production is specifically affected[3].

  3. Hypoplastic Anemia: This term describes a condition where there is a decrease in the number of blood-forming cells in the bone marrow, which can lead to reduced red blood cell production[7].

  4. Secondary Aplastic Anemia: This term may be used when the aplastic anemia is a result of another underlying condition or factor, such as autoimmune diseases, infections, or exposure to certain drugs or toxins[6].

  1. Anemia: A general term for a condition in which there is a deficiency of red blood cells or hemoglobin in the blood, leading to fatigue and weakness. APRCA is a specific type of anemia[5].

  2. Bone Marrow Failure: This broader term refers to the inability of the bone marrow to produce sufficient blood cells, which can include red blood cells, white blood cells, and platelets. APRCA is a specific manifestation of this failure[3].

  3. Autoimmune Hemolytic Anemia: While distinct from APRCA, this condition involves the immune system attacking red blood cells, leading to anemia. It is important to differentiate between these conditions as they have different underlying mechanisms and treatments[6].

  4. Cytopenia: This term refers to a reduction in the number of blood cells, which can include red blood cells, white blood cells, and platelets. APRCA specifically results in a reduction of red blood cells[5].

  5. Myelodysplastic Syndromes: These are a group of disorders caused by poorly formed or dysfunctional blood cells, which can sometimes lead to conditions like APRCA[7].

Conclusion

Understanding the alternative names and related terms for ICD-10 code D60.9 is crucial for accurate diagnosis and treatment of acquired pure red cell aplasia. These terms not only help in clinical settings but also enhance communication among healthcare professionals regarding the condition. If further information or clarification is needed on this topic, feel free to ask!

Diagnostic Criteria

Acquired pure red cell aplasia (PRCA) is a rare hematological disorder characterized by a significant reduction in red blood cell production due to the absence of erythroid progenitor cells in the bone marrow. The ICD-10 code D60.9 specifically refers to "Acquired pure red cell aplasia, unspecified," indicating that the condition is recognized but the specific cause or type is not detailed.

Diagnostic Criteria for Acquired Pure Red Cell Aplasia

Clinical Presentation

The diagnosis of acquired PRCA typically begins with a thorough clinical evaluation, which may include:

  • Symptoms: Patients often present with symptoms of anemia, such as fatigue, pallor, shortness of breath, and tachycardia. Severe cases may lead to more pronounced symptoms like dizziness or syncope due to low hemoglobin levels.
  • History: A detailed medical history is essential, including any recent infections, medications, autoimmune diseases, or exposure to toxins that could contribute to the condition.

Laboratory Findings

Key laboratory tests are crucial for diagnosing PRCA:

  • Complete Blood Count (CBC): A CBC will typically show a normocytic or macrocytic anemia with a low reticulocyte count, indicating inadequate red blood cell production.
  • Bone Marrow Biopsy: This is a definitive test for PRCA. The biopsy will reveal a marked reduction or absence of erythroid precursors while other hematopoietic lineages (white blood cells and platelets) may be normal or increased.
  • Serological Tests: Tests for viral infections (such as parvovirus B19, which is a common cause of PRCA) and autoimmune markers may be performed to identify underlying causes.

Exclusion of Other Conditions

It is important to rule out other causes of anemia and bone marrow suppression, which may include:

  • Aplastic Anemia: Characterized by a reduction in all blood cell types, not just red blood cells.
  • Myelodysplastic Syndromes: These can present with similar symptoms but involve dysplastic changes in the bone marrow.
  • Hemolytic Anemia: This condition involves the destruction of red blood cells, which can sometimes mimic PRCA.

Additional Considerations

  • Immunological Factors: In some cases, acquired PRCA may be associated with autoimmune disorders, necessitating further immunological evaluation.
  • Response to Treatment: The response to treatments such as corticosteroids or immunosuppressive therapy can also provide diagnostic clues, as some patients may improve with these interventions.

Conclusion

The diagnosis of acquired pure red cell aplasia (ICD-10 code D60.9) involves a combination of clinical assessment, laboratory testing, and exclusion of other hematological disorders. Understanding the underlying cause is crucial for effective management and treatment of the condition. If you suspect PRCA, it is advisable to consult a hematologist for a comprehensive evaluation and tailored diagnostic approach.

Treatment Guidelines

Acquired pure red cell aplasia (PRCA), classified under ICD-10 code D60.9, 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 idiopathic or secondary to various underlying causes, including autoimmune diseases, infections, and certain medications. Understanding the standard treatment approaches for this condition is crucial for effective management.

Overview of Acquired Pure Red Cell Aplasia

Definition and Causes

Acquired PRCA is defined as a selective reduction in red blood cell precursors in the bone marrow, leading to anemia. The causes can be diverse:
- Autoimmune Disorders: Conditions like systemic lupus erythematosus (SLE) can trigger PRCA.
- Infections: Viral infections, particularly parvovirus B19, are known to cause PRCA by infecting and destroying erythroid progenitor cells.
- Medications: Certain drugs, including some antibiotics and chemotherapeutic agents, can induce PRCA as a side effect.
- Malignancies: Some cancers, particularly lymphoproliferative disorders, can be associated with PRCA.

Standard Treatment Approaches

1. Identifying and Treating Underlying Causes

The first step in managing acquired PRCA is to identify and address any underlying conditions. This may involve:
- Discontinuing Offending Medications: If a drug is suspected to be the cause, it should be stopped immediately.
- Treating Infections: For viral infections like parvovirus B19, supportive care may be sufficient, as the body often clears the virus on its own.

2. Immunosuppressive Therapy

In cases where PRCA is associated with autoimmune disorders, immunosuppressive therapy may be indicated. Common agents include:
- Corticosteroids: Prednisone is often the first-line treatment to reduce immune-mediated destruction of erythroid progenitors.
- Other Immunosuppressants: Agents such as azathioprine or cyclosporine may be used in more severe cases or when corticosteroids are ineffective.

3. Erythropoiesis-Stimulating Agents

Erythropoietin (EPO) therapy can be beneficial in stimulating red blood cell production, especially in cases where the bone marrow is still capable of responding to erythropoietic signals. This approach is particularly useful in patients with chronic kidney disease or those who have not responded adequately to other treatments.

4. Blood Transfusions

In cases of severe anemia, blood transfusions may be necessary to manage symptoms and improve the patient's quality of life. However, this is typically a temporary measure and does not address the underlying cause of PRCA.

5. Bone Marrow Transplantation

For patients with severe, refractory PRCA, particularly those with associated malignancies or aplastic anemia, hematopoietic stem cell transplantation may be considered. This is a more aggressive approach and is usually reserved for specific cases.

6. Monitoring and Supportive Care

Regular monitoring of blood counts and overall health is essential. Supportive care, including nutritional support and management of complications related to anemia, is also important.

Conclusion

The management of acquired pure red cell aplasia (ICD-10 code D60.9) requires a comprehensive approach that includes identifying and treating underlying causes, employing immunosuppressive therapies, and providing supportive care. Each treatment plan should be tailored to the individual patient based on the severity of the condition and the presence of any underlying disorders. Regular follow-up and monitoring are crucial to ensure effective management and to adjust treatment as necessary.

Related Information

Description

  • Reduced red blood cell production
  • Failure of erythroid progenitor cells
  • Significant anemia in patients
  • Autoimmune diseases can cause PRCA
  • Infections like parvovirus B19 contribute
  • Medications and malignancies may be involved
  • Bone marrow biopsy reveals reduced erythroid precursors

Clinical Information

  • Significant reduction in red blood cell production
  • Failure of erythroid progenitor cells in bone marrow
  • Autoimmune disorders as a common underlying cause
  • Infections like parvovirus B19 can trigger PRCA
  • Certain medications lead to immune-mediated mechanisms
  • Hematological malignancies associated with PRCA
  • Fatigue and weakness due to anemia
  • Pallor resulting from reduced hemoglobin levels
  • Shortness of breath during physical exertion
  • Dizziness or lightheadedness due to low blood pressure

Approximate Synonyms

  • Erythroblastopenia
  • Aplastic Anemia
  • Hypoplastic Anemia
  • Secondary Aplastic Anemia
  • Anemia
  • Bone Marrow Failure
  • Autoimmune Hemolytic Anemia
  • Cytopenia
  • Myelodysplastic Syndromes

Diagnostic Criteria

  • Symptoms include fatigue and shortness of breath
  • Anemia with low reticulocyte count on CBC
  • Bone Marrow Biopsy shows reduced erythroid precursors
  • Absence or reduction of erythroid precursors in bone marrow
  • Ruling out Aplastic Anemia, Myelodysplastic Syndromes and Hemolytic Anemia
  • Presence of immunological factors may indicate autoimmune disorders

Treatment Guidelines

  • Identify and treat underlying causes
  • Discontinue offending medications immediately
  • Use corticosteroids for autoimmune-related PRCA
  • Try erythropoiesis-stimulating agents (ESA) therapy
  • Consider blood transfusions for severe anemia
  • Reserve bone marrow transplantation for refractory cases
  • Provide supportive care and monitoring

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It is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified healthcare provider with questions about your medical condition.