scholarly journals Acquired Pure Red Cell Aplasia (Prca) Responding to Erythropoietin Therapy in HIV Disease

2017 ◽  
Vol 2 (3) ◽  
Keyword(s):  
Myeloid Cells ◽  
Pure Red Cell Aplasia ◽  
Normocytic Anemia ◽  
Complete Disappearance ◽  
Red Cell ◽  
Hiv Disease ◽  
Red Cell Aplasia ◽  
Erythroid Precursors ◽  
Normal Production

Acquired pure red cell aplasia (PRCA) is characterized by the presence of an acquired normochromic, normocytic, anemia associated with a complete disappearance of reticulocytes and erythroid precursors in the marrow and normal production of myeloid cells and platelets. Mediators of suppression [1].

scholarly journals Immunophenotype of Erythroid Precursors in Patient with Pure Red Cell Aplasia (PRCA): Utility of Analysis of Erythroid Maturation

2021 ◽  
Vol 8 (5) ◽  
Author(s):  
Jerez J ◽  
◽  
Ocqueteau M ◽  
Keyword(s):  
Correct Diagnosis ◽  
Standard Of Care ◽  
Pure Red Cell Aplasia ◽  
Refractory Anemia ◽  
Red Cell ◽  
Red Cell Aplasia ◽  
Bone Marrow Histology ◽  
Erythroid Precursors ◽  
Normochromic Anemia ◽  
Erythroid Maturation

Pure Red Cell Aplasia (PRCA) is an infrequent disease [1,2], which usually presents as hypogenerative normochromic anemia, and is characterized by a significant decrease (including absence) of erythroid precursors [3]. Its etiology can be congenital or acquired, and its correct diagnosis requires exclusion of alternative cases of refractory anemia, so the bone marrow histology plays a crucial role. Myelodisplastic Syndromes (MDS) should always be considered in its differential diagnosis. The use of laboratory tools, specifically Flow Cytometry (FCM) is gained importance in the study of malignant and benign hematology pathologies. In MDS, FCM is not yet considered a standard of care, however it provides valuable information [4,5] and there are numerous publications and scores for its usual clinical use (for example Ogata score and RED-score [6,7]). In relation to the rise of FCM in MDS, enormous progress has been made in the description of the erythroid precursors immunophenotype [8-10]. An example of normal erythroid maturation is presented in Figure 1, showing proerythroblasts with immunophenotype CD71+ CD105+ CD117+, basophilic erythroblasts CD71+ CD105+ CD117-, polychromatophilic and orthochromatophilic erythroblasts CD71+ CD105- CD117- distinguishing by size in Forward Scatter (FSC) versus CD36 respectively. Characteristic maturation curve in CD117 versus CD105 analysis evidenced a predominance towards more mature erythroblasts.

scholarly journals Carbamazepine-Induced Pure Red Cell Aplasia

2018 ◽  
Vol 05 (01) ◽  
pp. 050-052 ◽  
Author(s):  
C. Mansoor ◽  
Laksmi Priya
Keyword(s):  
Bone Marrow ◽  
Rare Disease ◽  
Pure Red Cell Aplasia ◽  
Normocytic Anemia ◽  
Red Cell ◽  
Normal Bone Marrow ◽  
Hematologic Disorders ◽  
Normal Bone ◽  
Red Cell Aplasia ◽  
Carbamazepine Therapy

AbstractAntiepileptic therapy is associated with various hematologic disorders. Pure red cell aplasia (PRCA) is a rare disease that may be congenital or acquired. Severe normocytic anemia, reticulocytopenia, and absence of erythroblasts from an otherwise normal bone marrow should raise the suspicion of PRCA. A 32-year-old unmarried woman was admitted with fatigue for 4 months. She had been on carbamazepine therapy for 4 years (200 mg twice daily) for seizure disorder. On evaluation, she was diagnosed to have PRCA secondary to carbamazepine. We describe a patient with carbamazepine-induced PRCA that improved after discontinuation of the drug.

IL-3 increases marrow and peripheral erythroid precursors in chronic pure red cell aplasia presenting in childhood

1995 ◽  
Vol 89 (2) ◽  
pp. 413-416 ◽  
Author(s):  
Yves Bastion ◽  
Lydia Campos ◽  
Nora Roubi ◽  
Jacques Bienvenu ◽  
Pascale Felman ◽  
...  
Keyword(s):  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Red Cell Aplasia ◽  
Erythroid Precursors

scholarly journals Case Report: Pure Red Cell Aplasia due to Angioimmunoblastic T-Cell Lymphoma

2020 ◽  
Vol 13 (1) ◽  
pp. 76-78 ◽  
Author(s):  
Marina Vitorino ◽  
Filipa Nunes ◽  
Mariana Costa ◽  
Beatriz Porteiro ◽  
Alexys Reis Borges ◽  
...  
Keyword(s):  
Bone Marrow ◽  
T Cell ◽  
Cell Lymphoma ◽  
Bone Marrow Failure ◽  
Pure Red Cell Aplasia ◽  
T Cell Lymphoma ◽  
Normocytic Anemia ◽  
Red Cell ◽  
Angioimmunoblastic T Cell Lymphoma ◽  
Red Cell Aplasia

Pure red cell aplasia (PRCA) is a rare bone marrow failure characterized by a progressive normocytic anemia and reticulocytopenia without leukopenia and thrombocytopenia. It can be associated with various hematological disorders but exceedingly rarely with angioimmunoblastic T-cell lymphoma (AITL). We report the case of a 72-year-old woman with PRCA associated with AITL. The patient presented with severe anemia (hemoglobin 2.6 g/dL) and a low reticulocyte count 0.7%. Direct and indirect Coombs tests were positive. A CT scan of the chest, abdomen, and pelvis revealed multiple lymphadenopathies. A cervical lymph node biopsy was compatible with AITL. A bone marrow biopsy showed medullary involvement by AITL and a severe erythroid hypoplasia with a myeloid:erythroid ratio of 19.70. The patient was started on CHOP and after 6 cycles the PET scan confirmed complete remission.

scholarly journals A Patient with Acquired Pure Red Cell Aplasia Showing a Positive Antiglobulin Test and the Presence of Inhibitor against Erythroid Precursors.

2002 ◽  
Vol 41 (7) ◽  
pp. 589-592 ◽  
Author(s):  
Yasushi OKOSHI ◽  
Shigehiko IMAGAWA ◽  
Masato HIGUCHI ◽  
Chikashi YOSHIDA ◽  
Seiichi SHIMIZU ◽  
...  
Keyword(s):  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Red Cell Aplasia ◽  
Erythroid Precursors ◽  
Antiglobulin Test

scholarly journals UNUSUAL CASE OF ANAEMIA IN ADULT MALE

2020 ◽  
pp. 71-73
Author(s):  
Dnyaneshwar S Cheke ◽  
Isha Desai ◽  
Jitendra Ingole
Keyword(s):  
Bone Marrow ◽  
Marked Reduction ◽  
Unusual Case ◽  
Pure Red Cell Aplasia ◽  
Myeloproliferative Disorder ◽  
Red Cell ◽  
Red Cell Aplasia ◽  
Normochromic Anaemia ◽  
Erythroid Precursors ◽  
Normocytic Normochromic Anaemia

A case of Anaemia which was investigated and found to be a case of Acquired Pure Red Cell Aplasia. Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anaemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. It is commonly due to primary autoimmune or infection and secondary to myeloproliferative disorder. In this discussion we are presenting a case of normocytic normochromic anaemia which was admitted to our hospital after being investigated and treated several times outside. On extensive workup, we diagnosed the case as PRCA secondary to Thymoma and initiated the appropriate management.

Pure Red Cell Aplasia Caused by Azathioprine

2020 ◽  
Vol 20 (2) ◽  
pp. 164-165 ◽  
Author(s):  
Dimitris Kounatidis ◽  
Natalia Vallianou ◽  
Vasiliki Daskalaki ◽  
Christos Masaoutis ◽  
Evangelia Margellou ◽  
...  
Keyword(s):  
Vascular Diseases ◽  
Complete Recovery ◽  
Pure Red Cell Aplasia ◽  
Careful Consideration ◽  
Normocytic Anemia ◽  
Red Cell ◽  
Collagen Vascular Diseases ◽  
Red Cell Aplasia ◽  
Long Run ◽  
Wait And See

Background: Pure Red Cell Aplasia (PRCA) is a clinical entity comprising severe normochromic normocytic anemia, reticulocytopenia, erythroblastopenia in the bone marrow, with normal leukocyte and platelets count. PRCA can be classified into congenital and acquired, with the latter characterized as idiopathic or secondary to various infections, hematological malignancies, collagen vascular diseases, thymoma, and exposure to a variety of drugs and other chemical substances. Methods: Herein, we present a female patient, who presented with PRCA due to azathioprine treatment. Results: Prompt discontinuation of the drug together with red blood cells transfusions led to complete recovery in this young patient, without any addition of immunosuppressive regimen. Conclusion: We followed ‘the wait and see practice’ instead of administering immunosuppression to our patient, after careful consideration and extensive consultation with our hematologists. This ‘wait and see practice’ proved to be effective in the long run.

scholarly journals Parvovirus B19 infection in HIV-infected patients

2020 ◽  
Vol 92 (7) ◽  
pp. 100-103
Author(s):  
A. A. Petrenko ◽  
G. A. Dudina ◽  
N. V. Kremneva ◽  
A. V. Pivnik
Keyword(s):  
Viral Load ◽  
Antiretroviral Therapy ◽  
Parvovirus B19 ◽  
Pure Red Cell Aplasia ◽  
Intravenous Immunoglobulins ◽  
Normocytic Anemia ◽  
Red Cell ◽  
Test Results ◽  
Red Cell Aplasia ◽  
Laboratory Test Results

Here we provide a review of the literature and a description of our own clinical case. The patient was a 32-year-old woman who had been infected with HIV for 6 years without antiretroviral therapy. The test results showed CD4 87 cells/l, viral load 3750 copies/ml. Normochromic normocytic anemia and reticulocytopenia developed soon. In the myelogram, all erythroblasts were 0.5%. The viral load of parvovirus B19 DNA according to PCR was more than 9 million IU/ml. Pure red cell aplasia associated with parvovirus B19 was diagnosed. We started antiretroviral therapy with efavirenz, lamevudine and tenofovir. In addition to blood transfusions, we administered intravenous donor immunoglobulin with a dose increase from 5000 mg to 20 000 mg per day. After discontinuing of intravenous immunoglobulins, the laboratory test results were stable over the next 5 months: hemoglobin was more than 115 g/L, reticulocytes more than 3%, in the myelogram all erythroblasts were 21%. However, the elimination of parvovirus B19 wasnt achieved. The maximum decrease in viral load for parvovirus B19 was down to 720 IU/ml. A typical feature of the case was the lack of pure red cell aplasia of the bone marrow with the existing viral load of parvovirus B19. HIV infection progressed: 44 cells/l, viral load not determined. The case ended lethally.

scholarly journals Pure red cell aplasia: association with large granular lymphocyte leukemia and the prognostic value of cytogenetic abnormalities [see comments]

Blood ◽  
1996 ◽  
Vol 87 (7) ◽  
pp. 3000-3006 ◽  
Author(s):  
MQ Lacy ◽  
PJ Kurtin ◽  
A Tefferi
Keyword(s):  
T Cell ◽  
Immunosuppressive Therapy ◽  
Cyclosporine A ◽  
Immunosuppressive Agents ◽  
Pure Red Cell Aplasia ◽  
Red Cell ◽  
Cytogenetic Abnormalities ◽  
Red Cell Aplasia ◽  
Erythroid Precursors ◽  
Lgl Leukemia

From 1980 through 1994, we identified 47 adult patients with acquired pure red cell aplasia (median age, 64 years; range, 22 to 84 years). Associated clinical disorders included T-cell large granular lymphocytic (LGL) leukemia, thymoma, chronic lymphocytic leukemia, and non-Hodgkin's lymphoma. Review of bone marrow findings in 40 patients showed absence of erythroid precursors in 14 patients and rare pronormoblasts in 26. None had morphologic evidence of myelodysplasia. T-cell receptor gene rearrangement studies with Southern blot technique in 14 patients showed clonal rearrangements in nine. Karyotypic analyses performed in 28 patients showed clonal abnormalities in four. Overall, 28 of 47 patients (60%) responded to immunosuppressive therapy, but none were the patients with cytogenetic abnormalities. There was a trend toward superior response to immunosuppressive agents in the patients with T-cell LGL leukemia. Cyclophosphamide, with or without corticosteroids, was the most useful treatment agent. Cyclosporine A was effective for refractory disease. Neither the presence of an associated clinical disorder nor the existence of detectable erythroid precursors affected overall survival. We conclude that (1) T-cell LGL leukemia is the disorder most commonly associated with pure red cell aplasia, (2) the presence of clonal cytogenetic abnormality predicts poor response to immunosuppressive therapy, and (3) oral cyclophosphamide and cyclosporine A are effective treatment regimens.

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