Pure Red Cell Aplasia

Krantz, Sanford B.

doi:10.1111/j.1365-2141.1973.tb01710.x

Cited by 48 publications

(8 citation statements)

References 20 publications

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“…The Leser-Trelat sign, as defined by the sudden appearance and rapid increase in the number and size of keratosis with severe pruritis,2 is a rare manifestation of visceral cancer, a review in 1973 showing only 10 cases, including those from old European literature. Pure red blood cell aplasia has been most frequently seen in association with thymoma4 (accounting for approximately half of all cases) 6 and less freqently with l y m p h~m a ,~ chronic lymphocytic leukemia,' and carcinoma. * T h e association of pure red blood cell aplasia with carcinoma (without a co-existing thymoma) has, in fact, only been reported five timesx The occurrence of the Leser-Trelat sign, closely followed by pure red blood cell aplasia, in a patient with bile duct adenocarcinoma complicating longstanding ulcerative colitis, constitutes an unique case.…”

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confidence: 99%

Bile duct adenocarcinoma with leser-trelat sign and pure red blood cell aplasia

Lee

Clark

Thorpe

et al. 1980

Cancer

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A case of adenocarcinoma of the bile duct complicating longstanding ulcerative colitis and associated with the rapid onset and growth of multiple seborrheic keratosis (Leser-Trelat sign) and pure red blood cell aplasia is described. This is the first report of a case of the coexistence of the Leser-Trelat sign and pure red blood cell aplasia in a patient with a malignancy. Both manifestations have been doubted as truly paraneoplastic manifestations because of their rarity in clinical medicine. Their co-existence in the present case would support their associaton with malignancy.

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confidence: 99%

Bile duct adenocarcinoma with leser-trelat sign and pure red blood cell aplasia

Lee

Clark

Thorpe

et al. 1980

Cancer

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“…PRCA is a rare disorder characterized by normochromic, normocytic or slightly macrocytic anemia with reticulocytopenia and near-complete absence of erythroblasts in the bone marrow [13][14][15]. Diamond-Blackfan anemia is a rare congenital form of PRCA associated with hypoplastic anemia and a variety of cardiac, neurological, and craniofacial defects [15].…”

Section: Discussionmentioning

confidence: 99%

“…Diamond-Blackfan anemia is a rare congenital form of PRCA associated with hypoplastic anemia and a variety of cardiac, neurological, and craniofacial defects [15]. More commonly, PRCA manifests in the adult population and can be classified as primary (idiopathic) or secondary [14].…”

Section: Discussionmentioning

confidence: 99%

Pure red cell aplasia caused by ribavirin and interferon treatment

Skabelund

Hauser

Goist

2011

Clin J Gastroenterol

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Anemia is a common complication of hepatitis C (HCV), and antiviral treatment can further increase this risk. We present the case of a 59-year-old man with HCV treated with ribavirin and pegylated interferon alpha (INF-α) who presented with severe anemia. Two months after initiating treatment his hemoglobin dropped from 14.2 to 5.0 g/dL. There was no evidence of bleeding or hemolysis, and a bone marrow biopsy revealed pure red cell aplasia (PRCA). Evaluations for acute cytomegalovirus and parvovirus B19 were negative. There was no evidence of malignancy or thymoma. The INF-α and ribavirin treatment were determined to have caused the PRCA, and withdrawal of the medications led to PRCA remission. INF-α and ribavirin have become the standard treatment for HCV. While these medications offer a potential cure, they are often poorly tolerated due to frequent side effects including anemia. Patients who are receiving treatment with ribavirin and INF-α warrant close monitoring for development of anemia, and PRCA should be considered in all patients in whom reticulocytopenic anemia develops.

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“…The wide variation in age of onset, severity and response to therapy, and the irregularity of the associated phenotypic, biochemical and chromosomal abnormalities suggest that it is a syndrome of multiple causation. The possible pathogenetic mechanisms have been recently reviewed by Bloom (1972) and Krantz (1973). It is not uncommon to have two or more siblings affected and an autosomal recessive mode of inheritance may operate in these cases.…”

Section: Discussionmentioning

confidence: 99%