Ten year follow up of erythropoietin induced autoimmune pure red cell aplasia

As information on the clinical features and prognosis of thymoma complicated by pure red-cell aplasia is limited, follow-up data on thymoma patients who had a thymectomy between 1954 and 1999 were analyzed retrospectively. Six of 166 cases were complicated by pure red-cell aplasia. In 3 of these, the pure red-cell aplasia appeared after surgical intervention. Remission was observed in 2 patients who underwent extended thymectomy. The other 4 patients subsequently died from pure red-cell aplasia. The outcome in patients with pure red-cell aplasia was poorer than that in the entire group of patients with thymoma and in those with thymoma complicated by myasthenia gravis. The possible onset of pure red-cell aplasia after thymectomy should be kept in mind during follow-up.

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Long-term outcome of individuals with pure red cell aplasia and antierythropoietin antibodies in patients treated with recombinant epoetin: a follow-up report from the Research on Adverse Drug Events and Reports (RADAR) Project

Blood ◽

10.1182/blood-2005-02-0508 ◽

2005 ◽

Vol 106 (10) ◽

pp. 3343-3347 ◽

Cited By ~ 62

Author(s):

C. L. Bennett

Keyword(s):

Adverse Drug Events ◽

Pure Red Cell Aplasia ◽

Red Cell ◽

Term Outcome ◽

Long Term Outcome ◽

Red Cell Aplasia

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A Case of Systemic Lupus Erythematosus Presenting as Pure Red Cell Aplasia

International Journal of Hematology-Oncology and Stem Cell Research ◽

10.18502/ijhoscr.v15i2.6045 ◽

2021 ◽

Author(s):

Maria Saeed ◽

Fatima Sharif ◽

Maira Ijaz ◽

Shawana Kamran

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Pure Red Cell Aplasia ◽

Young Female ◽

Severe Anemia ◽

Red Cell ◽

Systemic Lupus ◽

Red Cell Aplasia ◽

Uncommon Condition

Pure red cell aplasia (PRCA) is an uncommon condition, which is rarely associated with Systemic Lupus Erythematosus (SLE). Prompt identification and management of the underlying SLE results in correction of anemia. We report the case of a young female who presented due to severe anemia since the last two years. The cause of her anemia on initial investigations was not elicited in these two years, during which response to hematinics was poor and she remained transfusion dependent. Bone marrow biopsy showed PRCA after which autoimmune workup revealed SLE. Subsequently, treatment of SLE with steroids led to normalization of hemoglobin levels within a follow-up period of three months.

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The Comparison of Myeloablative Versus Reduced Intensity Conditioning Regimen in ABO Mismatched Allogeneic Hematopoietic Cell Transplantation.

Blood ◽

10.1182/blood.v104.11.5146.5146 ◽

2004 ◽

Vol 104 (11) ◽

pp. 5146-5146

Author(s):

Pervin Topcuoglu ◽

Mutlu Arat ◽

Tugce Bolukbasi ◽

Ender Soydan ◽

Yesim Ozer ◽

...

Keyword(s):

Blood Group ◽

Acute Gvhd ◽

Pure Red Cell Aplasia ◽

Red Cell ◽

Reduced Intensity Conditioning ◽

Red Cell Aplasia ◽

Abo Incompatibility ◽

Reduced Intensity ◽

Related Mortality

Abstract Recently, in an effort to reduce the transplant related mortality, AHCT with reduced intensity conditioning (RIC) have been developed. It has been reported that patients receiving RIC from an ABO-mismatched donor had more transplant-associated complications compared to ablative AHCT (BMT2001;28: 315, Transfusion2002; 42:1293, Transfusion2003; 43: 1153). In this single center, retrospective, historical case-matched study, we aimed to analyze the influence of ABO-incompatibility on transplant-related morbidity and mortality between ablative and RIC. Between 1988 and 2003, 39 patients underwent AHCT with RIC and only 14 were ABO-incompatible. Ten patients with ABO-incompatibility and having a regular follow-up for blood group typing were evaluated for immuno-hematological complications, such as acute or delayed-type hemolysis (DTH), pure red cell aplasia (PRCA), thrombotic thrombocytopenic purpura (TTP) and early transplant-related complications, were compared to 20 case control recipients having matched pretransplant characteristics and similar follow up, but myeloablative regimen, either bone marrow (BM) or peripheral blood (PB) stem cells infused. Patients’ characteristics are shown in table below. All the recipients and donors underwent a detailed pretransplant work up and all the recipients were followed twice a week post transplant by a transfusion specialist according to guidelines (BMT, 2001;28:315). Median follow-up was 195 days (range, 51–538d). We did not observe any acute hemolysis, but 11 experienced DTH. No significant differences were encountered among the three groups in terms of DTH (p=0.356). In all recipients having a major ABO incompatibility, the blood group switched to donor type, but 50% of the patients with minor ABO-incompatibility still had either their antigen persistency or the appearance of donor-derived isoagglutinins. We observed mild (n=1, BM group) and severe pure red cell aplasia (n=1, RIC group) in two patients having a major ABO-incompatibility. TTP was developed in one patient with major ABO-incompatibility. In conclusion, we did not observe any difference between ablative AHCT and RIST in ABO incompatible patients in terms of immuno hematological complications in contrast to published case series. In addition, we could not show any negative impact of ABO-incompatibility on the severity of acute GVHD and the rate of early transplant related mortality. Table Conditioning Ablative Reduced Intensity Source (patients) PB (n=10) BM (n=10) PB (n=10) Gender (M/F) 2/8 6/4 5/5 Median age (range) 34 (16–45) 31 (17–46) 33 (21–47) Diagnosis CML (2), AML (7), other (1) CML (2), AML (6), other (2) CML (4), AML (1), other (5) ABO-incompatibility Major 5 6 5 Minor 5 4 5 Delayed type hemolysis 5 (50%) (22,23,55,90,120) 2 (20%) (18,27) 4 (40%)(20,130,165,188) RBC transfusion (100 days) 3 (0–15) 4 (0–10) 5 (0–10) Median days of the independence from pRBC 7 (0–31) 18 (0–41) 7 (0–28) Median follow-up (days) 195 (90–538) 194 (63–467) 200 (51–511) Acute GVHD (grade II–IV) n (%) 3 (30%) 4 (40%) 4 (40%) TRM 100, n (%) 1 (%10) 1 (%10) 1 (%10)

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A case of pure red cell aplasia: follow-up on different immunosuppressive regimens

The Clinical Investigator ◽

10.1007/bf00577754 ◽

1994 ◽

Vol 72 (12) ◽

pp. 1051-1055 ◽

Cited By ~ 2

Author(s):

M. Möller ◽

H. Walter ◽

H. Brass ◽

W. Queiβer

Keyword(s):

Pure Red Cell Aplasia ◽

Red Cell ◽

Red Cell Aplasia

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The pathophysiology of pure red cell aplasia: implications for therapy

Blood ◽

10.1182/blood.v87.11.4831.bloodjournal87114831 ◽

1996 ◽

Vol 87 (11) ◽

pp. 4831-4838 ◽

Cited By ~ 116

Author(s):

RJ Charles ◽

KM Sabo ◽

PG Kidd ◽

JL Abkowitz

Keyword(s):

Drug Exposure ◽

Mononuclear Cells ◽

Parvovirus B19 ◽

Pure Red Cell Aplasia ◽

Immunosuppressive Drugs ◽

Red Cell ◽

Red Cell Aplasia ◽

Chi Square Analysis

To determine the utility of marrow culture in defining the natural history and therapeutic response of pure red cell aplasia we have studied 37 patients. Patients were evaluated at the University of Washington before specific therapies (n = 21) or at the time of treatment failure in = 16). Evaluation included a medical and drug exposure history, a physical examination, a chest x-ray or computed tomography to rule out thymoma, lymphocyte immunophenotype studies, anti-nuclear antibody and rheumatoid factor determinations, marrow cytogenetics, and marrow progenitor cell cultures. Retrospective Southern analyses to detect human parvovirus B19 was performed in the 27 patients for whom sera was stored. Clinical follow-up was obtained to document therapeutic responses. Normal burst forming unit-erythroid (BFU-E) growth (>30 bursts/10(5) marrow mononuclear cells [MMNC]) in culture proved an outstanding predictor of clinical response, as 27 of 29 individuals with normal frequencies of erythroid bursts in culture responded to immunomodulating therapies (sensitivity 96%, specificity 78%, predictive value 93%, P = .0001 with two-tailed chi square analysis). Overall, 28 patients responded to either immunomodulating therapies or drug withdrawal. Twenty-four patients obtained a normal hematocrit (complete response [CR] and 4 additional patients became transfusion independent (partial response). Although responding patients often required several therapies, 20 of 24 (83%) patients who obtained a CR have sustained a normal hematocrit without maintenance therapy at the time of last follow-up (median 5 years). In contrast, of 8 patients with poor in vitro BFU-E growth (<6 bursts/10(5) MMNC), 7 failed to respond to any therapy and all died (median survival time 17 months). Our data suggest that in individuals, from whom BFU-E mature appropriately in culture, immunosuppressive drugs should be used sequentially until a CR is obtained and a durable remission is the expected outcome.

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Studies on pure red cell aplasia. XI. Results of immunosuppressive treatment of 37 patients

Blood ◽

10.1182/blood.v63.2.277.277 ◽

1984 ◽

Vol 63 (2) ◽

pp. 277-286 ◽

Cited By ~ 89

Author(s):

DA Clark ◽

EN Dessypris ◽

SB Krantz

Keyword(s):

Antithymocyte Globulin ◽

Immunosuppressive Treatment ◽

Pure Red Cell Aplasia ◽

Additional Treatment ◽

Cytotoxic Drugs ◽

Red Cell ◽

Red Cell Aplasia ◽

Effective Form ◽

Immune Manipulation

Abstract Thirty-seven patients with pure red cell aplasia (PRCA) were seen between 1966 and 1982. Ten patients had accompanying diseases described in association with PRCA, while the remainder had primary PRCA. All but two patients were treated with some form of immune manipulation, including corticosteroids, cytotoxic drugs, antithymocyte globulin, splenectomy, thymectomy, and plasmapheresis. Twenty-three patients (66%) had a remission induced by immunosuppression. In addition, there were 5 spontaneous remissions (14%). Cytotoxic drugs administered in combination with corticosteroids were the most effective form of treatment, producing 18/32 remissions (56%). Twelve of these remissions were in patients resistant to corticosteroids or in patients who had relapsed while taking them. Thirteen of the 23 patients in whom remissions were induced and one-fifth of the patients with spontaneous remissions have relapsed to date. However, with additional treatment, a second remission was induced in 10/13. Fifty-four percent of the patients with induced remissions remained transfusion-free during most of the follow-up period. Median survival in patients with primary PRCA was greater than 10 yr, whereas in patients with secondary PRCA, it was 4 yr. Infection was a major cause of morbidity and mortality. This study demonstrates the value of a variety of immunosuppressive treatments of patients with PRCA.

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Acquired pure red cell aplasia associated with lymphoproliferative disease of granular T lymphocytes

Blood ◽

10.1182/blood.v98.2.483 ◽

2001 ◽

Vol 98 (2) ◽

pp. 483-485 ◽

Cited By ~ 64

Author(s):

Ronald S. Go ◽

Chin-Yang Li ◽

Ayalew Tefferi ◽

Robert L. Phyliky

Keyword(s):

T Lymphocytes ◽

Clinical Course ◽

Significant Proportion ◽

Pure Red Cell Aplasia ◽

Lymphoproliferative Disease ◽

Red Cell ◽

General Group ◽

Red Cell Aplasia ◽

Treatment Responses

Acquired pure red cell aplasia (PRCA) can be associated with lymphoproliferative disease of granular T lymphocytes (T-LDGL), also known as T-cell large granular lymphocyte leukemia. Fifteen adult patients with PRCA associated with T-LDGL comprise this study. Neutropenia and rheumatoid arthritis were uncommon. All patients responded to immunosuppressive therapy. The 2 most commonly used treatments were prednisone and cyclophosphamide ± corticosteroids, producing overall response rates of 50% and 60%, respectively. Treatment with cyclophosphamide was associated with a more durable remission (median, 60 versus 7.5 months). After a median follow-up of 67 months, 2 patients died of treatment-related complications, one from myelodysplasia and another from cyclosporine-induced renal failure. The clinical course and treatment responses of PRCA associated with T-LDGL in this series were similar to the general group of PRCA. Because T-LDGL is frequently underdiagnosed, it is likely that a significant proportion of idiopathic or primary PRCA is in fact secondary to T-LDGL.

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Studies on pure red cell aplasia. XI. Results of immunosuppressive treatment of 37 patients

Blood ◽

10.1182/blood.v63.2.277.bloodjournal632277 ◽

1984 ◽

Vol 63 (2) ◽

pp. 277-286 ◽

Cited By ~ 2

Author(s):

DA Clark ◽

EN Dessypris ◽

SB Krantz

Keyword(s):

Antithymocyte Globulin ◽

Immunosuppressive Treatment ◽

Pure Red Cell Aplasia ◽

Additional Treatment ◽

Cytotoxic Drugs ◽

Red Cell ◽

Red Cell Aplasia ◽

Effective Form ◽

Immune Manipulation

Thirty-seven patients with pure red cell aplasia (PRCA) were seen between 1966 and 1982. Ten patients had accompanying diseases described in association with PRCA, while the remainder had primary PRCA. All but two patients were treated with some form of immune manipulation, including corticosteroids, cytotoxic drugs, antithymocyte globulin, splenectomy, thymectomy, and plasmapheresis. Twenty-three patients (66%) had a remission induced by immunosuppression. In addition, there were 5 spontaneous remissions (14%). Cytotoxic drugs administered in combination with corticosteroids were the most effective form of treatment, producing 18/32 remissions (56%). Twelve of these remissions were in patients resistant to corticosteroids or in patients who had relapsed while taking them. Thirteen of the 23 patients in whom remissions were induced and one-fifth of the patients with spontaneous remissions have relapsed to date. However, with additional treatment, a second remission was induced in 10/13. Fifty-four percent of the patients with induced remissions remained transfusion-free during most of the follow-up period. Median survival in patients with primary PRCA was greater than 10 yr, whereas in patients with secondary PRCA, it was 4 yr. Infection was a major cause of morbidity and mortality. This study demonstrates the value of a variety of immunosuppressive treatments of patients with PRCA.

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