Acquired pure red cell aplasia - Clinicals, Diagnosis, and Management

Hematology

Clinicals - History

Fact Explanation
An underlying predisposing condition Most of the times pure red cell aplasia is associated with autoimmune conditions, but there have been strong relationships to thymoma, chronic kidney disease, Parvovirus B19 infection, hematological malignancies and anti-erythropoietin antibodies. Out of the drugs (except erythropoietin, of course, since mentioned above), phenytoin, azathioprine, and isoniazid have been recognized as common drugs that cause pure red cell aplasia. An underlying predisposing condition
Most of the times pure red cell aplasia is associated with autoimmune conditions, but there have been strong relationships to thymoma, chronic kidney disease, Parvovirus B19 infection, hematological malignancies and anti-erythropoietin antibodies. Out of the drugs (except erythropoietin, of course, since mentioned above), phenytoin, azathioprine, and isoniazid have been recognized as common drugs that cause pure red cell aplasia.
Features of severe anemia Severe exertional dyspnea, chest pain on exertion, fatigue and lethargy are major symptoms of severe anemia. The symptoms of leucocytopenia (i.e. frequent bacterial infections, poor wound healing ) or thrombocytopenia (i.e. mucosal bleeding, petichial hemorrhages) are not to be seen in pure red cell aplasia. Features of severe anemia
Severe exertional dyspnea, chest pain on exertion, fatigue and lethargy are major symptoms of severe anemia. The symptoms of leucocytopenia (i.e. frequent bacterial infections, poor wound healing ) or thrombocytopenia (i.e. mucosal bleeding, petichial hemorrhages) are not to be seen in pure red cell aplasia.
Acutely presenting in children Acute acquired form is predominantly present in children. Acutely presenting in children
Acute acquired form is predominantly present in children.
Chronically presenting in adults Chronic form is predominantly present in adults. Chronically presenting in adults
Chronic form is predominantly present in adults.
Features of acute heart failure i.e. orthopnea, paroxysmal nocturnal dyspnea, exertional intolerance. Due to high output cardiac failure. Features of acute heart failure
i.e. orthopnea, paroxysmal nocturnal dyspnea, exertional intolerance. Due to high output cardiac failure.

Clinicals - Examination

Fact Explanation
Pallor Pallor in the mucus membranes, conjunctiva and palms/soles is a prominent feature of anemia. Absence of pallor does not rule out the presence of anemia. Pallor
Pallor in the mucus membranes, conjunctiva and palms/soles is a prominent feature of anemia. Absence of pallor does not rule out the presence of anemia.
Signs of acute heart failure Ankle edema, bilateral fine crepitations of the lung, increased jugular venous pressure. Due to high output cardiac failure. Signs of acute heart failure
Ankle edema, bilateral fine crepitations of the lung, increased jugular venous pressure. Due to high output cardiac failure.

Investigations - Diagnosis

Fact Explanation
Complete blood count Hemoglobin and red blood cell count is low, but other cell lineages are not affected. The MCV and MCH are normal. Complete blood count
Hemoglobin and red blood cell count is low, but other cell lineages are not affected. The MCV and MCH are normal.
Blood picture There is a normocytic normochromic anemia. Blood picture
There is a normocytic normochromic anemia.
Bone marrow biopsy for histology Shows hypoplasia of the erythroid series of cells. Bone marrow biopsy for histology
Shows hypoplasia of the erythroid series of cells.
Echocardiography To exclude or quantify cardiac failure. Echocardiography
To exclude or quantify cardiac failure.
CT scan neck To exclude thymoma. CT scan neck
To exclude thymoma.

Management - Supportive

Fact Explanation
Educate the patient Educate the patient about the nature and course of the disease, and the necessity of adherence to the treatments. Educate the patient
Educate the patient about the nature and course of the disease, and the necessity of adherence to the treatments.
Treat the underlying disease There are so many associated disease and predisposing conditions, drugs studied in relation to pure red cell aplasia. their diagnosis and management should be done as indicated. i.e. surgical removal of thymomas, optimizing renal functions in chronic kidney disease. Treat the underlying disease
There are so many associated disease and predisposing conditions, drugs studied in relation to pure red cell aplasia. their diagnosis and management should be done as indicated. i.e. surgical removal of thymomas, optimizing renal functions in chronic kidney disease.
Stop the offending drugs if any The known drugs that can be stopped should be stopped. i.e. erythropietin,phenytoin, azathioprin. Other drug classes should be considered instead. Stop the offending drugs if any
The known drugs that can be stopped should be stopped. i.e. erythropietin,phenytoin, azathioprin. Other drug classes should be considered instead.

Management - Specific

Fact Explanation
Red cell concentrate transfusion To correct anemia rapidly. Some patients might exhibit transfusion dependence, which might resolve spontaneously or with danazol. Red cell concentrate transfusion
To correct anemia rapidly. Some patients might exhibit transfusion dependence, which might resolve spontaneously or with danazol.
Danazol Maintains remission in refractory cases. Danazol
Maintains remission in refractory cases.
Corticosteroids First line treatment, relapses are common though. Corticosteroids
First line treatment, relapses are common though.
Other immunosuppressive drugs In some studies they have proven that up to 80% of the patients can relapse after corticosteroids. The other drugs that can be used are cyclosporine A, other cytotoxic immunosuppressive dugs alemtuzumab and rituximab. Other immunosuppressive drugs
In some studies they have proven that up to 80% of the patients can relapse after corticosteroids. The other drugs that can be used are cyclosporine A, other cytotoxic immunosuppressive dugs alemtuzumab and rituximab.

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