Pure Red Cell Aplasia (PRCA) is a rare disorder which causes anemia because the bone marrow is not able to produce mature red blood cells in the body.
In 1922, Kaznelson first introduced PRCA disorder which is different from disorder called aplastic anemia is occurs due to pancytopenia.
Erythroblasts are largely absent in bone marrow hence white blood cell and platelet production is remain normal.
Epidemiology
Pure red cell aplasia (PRCA) is a rare disorder and is seen in idiopathic form as one of the most common types. In transient and reversible cases in childhood and adults are underreported due to medications and infections.
It has two types where primary PRCA occurs without any symptoms and in secondary PRCA is linked to conditions like autoimmune diseases.
Anatomy
Pathophysiology
PRCA impairs with erythropoiesis i.e. it forms red blood cells which affects various stages of erythroid including progenitor development, differentiation, and maturation of previous cells.
Diamond-Blackfan syndrome is a rare congenital PRCA and is detected at time of birth or later stage of the first 18 months from birth.
PRCA is a condition which is caused by T-cell inhibition of marrow erythroid cells.
Etiology
Parvovirus B19 infection is the primary cause of acute transient PRCA cases which stays longer in immunocompromised patients.
Certain medications or toxins can cause PRCA which suppresses bone marrow or initiates immune-mediated reactions.
PRCA is linked to various autoimmune disorders where the immune system of body is mistakenly attacks on its own tissues.
Genetics
Prognostic Factors
Transient erythroblastopenia disorders in children and adults are benign with good prognosis, while secondary PRCA’s prognosis depends on the underlying condition’s course.
Severe anemia can lead to more severe symptoms and complications in patients and patients those who gives good response to treatment, such as immunosuppressive therapy in such case it may achieve remission and experience long-term improvement.
Clinical History
PRCA is mostly seen in adults from the age group of middle-aged to older individuals.
Physical Examination
Assessment of Vital Signs
Cardiovascular Examination
Respiratory Examination
Neurological Examination
Age group
Associated comorbidity
Associated activity
Acuity of presentation
Patients sometimes may suddenly experience symptoms like fatigue, weakness, and shortness of breath.
In patients with anemia, as severity can vary significantly in mild to severe cases which required immediate medical attention and treatment.
Differential Diagnoses
Secondary Causes of Anemia
Hemolytic Anemias
Myelodysplastic Syndromes
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
The identification of the main cause of PRCA is vital for making informed treatment choices.
In primary cases of PRCA, the physician preferred immunosuppressive therapy as it inhibit autoimmune responses and restore normal red blood cell production in the body of patient.
Supportive care and preventive measures along with management of complication should be carried out to improve the overall health and quality of life of patients.
Appointments with medical physicians and preventing recurrence of disorder is an ongoing life-long process.
Physician should encourage the patients about good hygiene practices and how to minimize exposure to infectious agents which reduces the risk of anemia.
Nutritional counselling plays vital role in recovery process of patient as it promotes the nutritional diet which increase red blood cell production.
To deal with fatigue and stress issues patient should follow occupational or physical therapy which is useful to manage fatigue and maintain functional stability.
Use of Corticosteroids for treatment of Pure Red Cell Aplasia
Prednisone: It has immunosuppressive properties which inhibits the production and activity of inflammatory cytokines. It suppresses immune-mediated destruction of red blood cells in the bone marrow.
Use of immunosuppressive Agents for treatment of Pure Red Cell Aplasia
Cyclophosphamide: It is nitrogen mustard chemical agent, and its active metabolites are involved in cross-linking of DNA which can damage cell growth of both normal and neoplastic cells.
Azathioprine: It is an immunosuppressive agent which interferes with DNA synthesis and inhibits lymphocyte proliferation.
Thymectomy is a surgical procedure in which surgeon remove the thymus gland. It is a potential treatment option in patients with those who are diagnosed with thymoma.
Thymectomy may be performed in thymoma patients, but not with  normal-sized thymus patients and in study it is observed that about 30% of thymomas patients respond positively to this procedure.
use-of-phases-in-managing-pure-red-cell-aplasia
In the initial diagnosis phase, the physician assess symptoms related to PRCA which is followed by diagnostic tests, including complete blood count, peripheral blood smear, and erythropoietin levels to confirm the diagnosis of anemia.
The next phase is supportive care and preventive measures where all necessary steps should be taken by physiotherapists to enhance the quality of life of the patient.
The regular follow-up visits with medical physician is required to check the improvement of patients and newly observed complaints.
Pure Red Cell Aplasia (PRCA) is a rare disorder which causes anemia because the bone marrow is not able to produce mature red blood cells in the body.
In 1922, Kaznelson first introduced PRCA disorder which is different from disorder called aplastic anemia is occurs due to pancytopenia.
Erythroblasts are largely absent in bone marrow hence white blood cell and platelet production is remain normal.
Pure red cell aplasia (PRCA) is a rare disorder and is seen in idiopathic form as one of the most common types. In transient and reversible cases in childhood and adults are underreported due to medications and infections.
It has two types where primary PRCA occurs without any symptoms and in secondary PRCA is linked to conditions like autoimmune diseases.
PRCA impairs with erythropoiesis i.e. it forms red blood cells which affects various stages of erythroid including progenitor development, differentiation, and maturation of previous cells.
Diamond-Blackfan syndrome is a rare congenital PRCA and is detected at time of birth or later stage of the first 18 months from birth.
PRCA is a condition which is caused by T-cell inhibition of marrow erythroid cells.
Parvovirus B19 infection is the primary cause of acute transient PRCA cases which stays longer in immunocompromised patients.
Certain medications or toxins can cause PRCA which suppresses bone marrow or initiates immune-mediated reactions.
PRCA is linked to various autoimmune disorders where the immune system of body is mistakenly attacks on its own tissues.
Transient erythroblastopenia disorders in children and adults are benign with good prognosis, while secondary PRCA’s prognosis depends on the underlying condition’s course.
Severe anemia can lead to more severe symptoms and complications in patients and patients those who gives good response to treatment, such as immunosuppressive therapy in such case it may achieve remission and experience long-term improvement.
PRCA is mostly seen in adults from the age group of middle-aged to older individuals.
Assessment of Vital Signs
Cardiovascular Examination
Respiratory Examination
Neurological Examination
Patients sometimes may suddenly experience symptoms like fatigue, weakness, and shortness of breath.
In patients with anemia, as severity can vary significantly in mild to severe cases which required immediate medical attention and treatment.
Secondary Causes of Anemia
Hemolytic Anemias
Myelodysplastic Syndromes
The identification of the main cause of PRCA is vital for making informed treatment choices.
In primary cases of PRCA, the physician preferred immunosuppressive therapy as it inhibit autoimmune responses and restore normal red blood cell production in the body of patient.
Supportive care and preventive measures along with management of complication should be carried out to improve the overall health and quality of life of patients.
Appointments with medical physicians and preventing recurrence of disorder is an ongoing life-long process.
Hematology
Physician should encourage the patients about good hygiene practices and how to minimize exposure to infectious agents which reduces the risk of anemia.
Nutritional counselling plays vital role in recovery process of patient as it promotes the nutritional diet which increase red blood cell production.
To deal with fatigue and stress issues patient should follow occupational or physical therapy which is useful to manage fatigue and maintain functional stability.
Hematology
Prednisone: It has immunosuppressive properties which inhibits the production and activity of inflammatory cytokines. It suppresses immune-mediated destruction of red blood cells in the bone marrow.
Hematology
Cyclophosphamide: It is nitrogen mustard chemical agent, and its active metabolites are involved in cross-linking of DNA which can damage cell growth of both normal and neoplastic cells.
Azathioprine: It is an immunosuppressive agent which interferes with DNA synthesis and inhibits lymphocyte proliferation.
Hematology
Thymectomy is a surgical procedure in which surgeon remove the thymus gland. It is a potential treatment option in patients with those who are diagnosed with thymoma.
Thymectomy may be performed in thymoma patients, but not with  normal-sized thymus patients and in study it is observed that about 30% of thymomas patients respond positively to this procedure.
Hematology
In the initial diagnosis phase, the physician assess symptoms related to PRCA which is followed by diagnostic tests, including complete blood count, peripheral blood smear, and erythropoietin levels to confirm the diagnosis of anemia.
The next phase is supportive care and preventive measures where all necessary steps should be taken by physiotherapists to enhance the quality of life of the patient.
The regular follow-up visits with medical physician is required to check the improvement of patients and newly observed complaints.
Pure Red Cell Aplasia (PRCA) is a rare disorder which causes anemia because the bone marrow is not able to produce mature red blood cells in the body.
In 1922, Kaznelson first introduced PRCA disorder which is different from disorder called aplastic anemia is occurs due to pancytopenia.
Erythroblasts are largely absent in bone marrow hence white blood cell and platelet production is remain normal.
Pure red cell aplasia (PRCA) is a rare disorder and is seen in idiopathic form as one of the most common types. In transient and reversible cases in childhood and adults are underreported due to medications and infections.
It has two types where primary PRCA occurs without any symptoms and in secondary PRCA is linked to conditions like autoimmune diseases.
PRCA impairs with erythropoiesis i.e. it forms red blood cells which affects various stages of erythroid including progenitor development, differentiation, and maturation of previous cells.
Diamond-Blackfan syndrome is a rare congenital PRCA and is detected at time of birth or later stage of the first 18 months from birth.
PRCA is a condition which is caused by T-cell inhibition of marrow erythroid cells.
Parvovirus B19 infection is the primary cause of acute transient PRCA cases which stays longer in immunocompromised patients.
Certain medications or toxins can cause PRCA which suppresses bone marrow or initiates immune-mediated reactions.
PRCA is linked to various autoimmune disorders where the immune system of body is mistakenly attacks on its own tissues.
Transient erythroblastopenia disorders in children and adults are benign with good prognosis, while secondary PRCA’s prognosis depends on the underlying condition’s course.
Severe anemia can lead to more severe symptoms and complications in patients and patients those who gives good response to treatment, such as immunosuppressive therapy in such case it may achieve remission and experience long-term improvement.
PRCA is mostly seen in adults from the age group of middle-aged to older individuals.
Assessment of Vital Signs
Cardiovascular Examination
Respiratory Examination
Neurological Examination
Patients sometimes may suddenly experience symptoms like fatigue, weakness, and shortness of breath.
In patients with anemia, as severity can vary significantly in mild to severe cases which required immediate medical attention and treatment.
Secondary Causes of Anemia
Hemolytic Anemias
Myelodysplastic Syndromes
The identification of the main cause of PRCA is vital for making informed treatment choices.
In primary cases of PRCA, the physician preferred immunosuppressive therapy as it inhibit autoimmune responses and restore normal red blood cell production in the body of patient.
Supportive care and preventive measures along with management of complication should be carried out to improve the overall health and quality of life of patients.
Appointments with medical physicians and preventing recurrence of disorder is an ongoing life-long process.
Hematology
Physician should encourage the patients about good hygiene practices and how to minimize exposure to infectious agents which reduces the risk of anemia.
Nutritional counselling plays vital role in recovery process of patient as it promotes the nutritional diet which increase red blood cell production.
To deal with fatigue and stress issues patient should follow occupational or physical therapy which is useful to manage fatigue and maintain functional stability.
Hematology
Prednisone: It has immunosuppressive properties which inhibits the production and activity of inflammatory cytokines. It suppresses immune-mediated destruction of red blood cells in the bone marrow.
Hematology
Cyclophosphamide: It is nitrogen mustard chemical agent, and its active metabolites are involved in cross-linking of DNA which can damage cell growth of both normal and neoplastic cells.
Azathioprine: It is an immunosuppressive agent which interferes with DNA synthesis and inhibits lymphocyte proliferation.
Hematology
Thymectomy is a surgical procedure in which surgeon remove the thymus gland. It is a potential treatment option in patients with those who are diagnosed with thymoma.
Thymectomy may be performed in thymoma patients, but not with  normal-sized thymus patients and in study it is observed that about 30% of thymomas patients respond positively to this procedure.
Hematology
In the initial diagnosis phase, the physician assess symptoms related to PRCA which is followed by diagnostic tests, including complete blood count, peripheral blood smear, and erythropoietin levels to confirm the diagnosis of anemia.
The next phase is supportive care and preventive measures where all necessary steps should be taken by physiotherapists to enhance the quality of life of the patient.
The regular follow-up visits with medical physician is required to check the improvement of patients and newly observed complaints.
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