Pure red cell aplasia in a lung transplant patient

Aplastic anemia secondary to infection by parvovirus B19 is normally an extremely rare problem in patients with no prior history. However, the presence of certain risks, such as receiving chronic immunosuppressant therapy, may facilitate its appearance. Very few cases have been published concerning red cell aplasia due to parvovirus B19 infection in patients receiving a transplanted lung. We report the case of a 24-year-old woman with cystic fibrosis who had received a double lung transplant. The patient developed red cell aplasia secondary to parvovirus B19 infection; severe anemia requiring multiple transfusions. Five days of intravenous immunoglobulin therapy resolved the anemia. We discuss the difficulty of serological diagnosis in such cases, the importance of using techniques that identify the virus and taking measures that may prevent recurrence.     

Pure red cell aplasia in a patient with systemic lupus erythematosus

Pure red cell aplasia developed in a female patient with systemic lupus erythematosus (SLE). Erythroid colony growth was assessed in semisolid medium culture of bone marrow obtained from a normal donor and cultured in the presence of normal and patient sera. Colony forming units of erythropoiesis and burst forming units of erythropoiesis obtained from a normal donor were inhibited in the presence of patient sera. Our findings support the concept that circulating inhibitors might influence the proliferation of erythroid progenitor cells and erythroid aplasia may be an immunologically mediated syndrome.     

Pure red cell aplasia in a patient with chronic granulocytic leukaemia treated with interferon-alpha

Interferon-alpha has been shown to improve survival in patients with chronic granulocytic leukaemia, therefore it is increasingly becoming part of the standard treatment of this condition. Interferon has a wide variety of side-effects. Pure red cell aplasia has been reported in a few cases of chronic granulocytic leukaemia but this usually heralds the onset of the transformation to the acute phase. This paper reports a possible, and not previously reported, side-effect of interferon-alpha in a patient with chronic granulocytic leukaemia.     

Pure red cell aplasia associated with fenoprofen

A 69-year-old man developed pure red cell aplasia after taking fenoprofen for ten months. The erythroid defect fully reversed after the drug was discontinued and could not be attributed to the patient's previously treated lung carcinoma. This case represents the third example of erythroid aplasia associated with an anti-inflammatory agent and the first instance due to fenoprofen.     

Pure red cell aplasia associated with thymoma

A case of thymoma with mixed spindle and lymphocytic variety along with acquired, secondary, chronic pure red cell aplasia is an uncommon entity. The presented case is a case of anterior mediastinal tumour with marked anemia. On histopathologic and hematological examinations, it proved to be a case of thymoma along with pure red cell aplasia. High index of suspicion, bone marrow examination, radiological including CT scan examination, total thymectomy after preparation with repeated blood transfusion remains the mainstay of treatment. Prolonged corticosteroid therapy leads to remission for upto ten months after operative intervention.     

Pure red cell aplasia due to parvovirus B19 in a patient treated with rituximab

Rituximab is a chimeric monoclonal antibody directed against CD20 and used in the treatment of B-cell non-Hodgkin's lymphoma. Due to its ability to deplete B lymphocytes, rituximab can interfere with humoral immunity, causing it to be suppressed for several months after treatment. The reported case depicts a serious consequence of this effect of rituximab therapy: pure red cell aplasia resulting from chronic parvovirus B19 infection. The point of interest in this case is not only the association between rituximab therapy and pure red cell aplasia, but the diagnostic and therapeutic utility of the knowledge of parvovirus B19 as the likely etiologic link between the two. Given the known efficacy of intravenous immunoglobulin (IVIg) in the treatment of chronic parvovirus B19 infection, this therapy can cure some of these patients and successfully render most others transfusion-independent until recovery of their own humoral immune system.     

Pure red cell aplasia and lupus

OBJECTIVE: To review the clinical and laboratory features of all reported patients with systemic lupus erythematosus (SLE) and pure red cell aplasia (PRCA). METHODS: In addition to our patient, we identified cases reported during the years 1966-2000 by searching the MEDLINE literature (Winspirs). Clinical and laboratory features were compared with those reported in large series of patients with SLE but without PRCA. RESULTS: Twenty-three additional cases were identified. In most cases, SLE was diagnosed either before or concomitantly with the diagnosis of PRCA. The clinical and laboratory features were not significantly different from those reported in large series of patients with SLE, except for less pleuritis and a trend toward less proteinuria, hallucinations, thrombopenia, and leukopenia. The natural history of PRCA and SLE was similar to that reported for PRCA alone. The disease responded to prednisone in the majority of cases, but patients frequently remained steroid dependent. CONCLUSIONS: The association between SLE and PRCA is rare. The clinical and laboratory features of SLE in such patients are similar to SLE patients without PRCA with the exception of a decreased frequency of pleuritis. Response to treatment of PRCA in those with SLE is similar to patients with PRCA but without SLE.     

Pure red cell aplasia in rheumatoid arthritis

We report a patient with longstanding rheumatoid arthritis (RA) who developed pure red cell aplasia. This condition is a rare complication of RA. Our patient recovered, apparently in response to treatment with corticosteroids and cyclophosphamide followed by azathioprine.     

Pure red cell aplasia induced only by intravenous administration of recombinant human erythropoietin

Antibody (Ab)-mediated pure red cell aplasia (PRCA) is a rare but important side effect in patients with chronic kidney disease who receive recombinant human erythropoietin (rhEPO). Ab-mediated PRCA was first reported in the 1990s, and the incidence subsequently increased and reached a peak in 2001. After improvements in rhEPO products and the administration route, the incidence was reduced by 90%, and now Ab-mediated PRCA only develops in a limited number of patients who receive rhEPO subcutaneously for a long period. We describe here the clinical course of one such rare patient with Ab-mediated PRCA. The patient was a 70-year-old man with chronic renal failure secondary to diabetic nephropathy. He had not received rhEPO therapy before the initiation of hemodialysis. He started hemodialysis and began to receive rhEPO therapy intravenously. Three months later, his hemoglobin level started declining and he became transfusion dependent. A diagnosis of Ab-mediated PRCA was made by bone marrow examination and detection of anti-EPO Abs. He was successfully treated with cyclosporine and became independent of blood transfusions. This case is a reminder that vigilance is required regarding the development of Ab-mediated PRCA upon rhEPO therapy, regardless of the administration route.