Idiopathic pure red cell aplasia: first report on CD8 positive lymphocytosis in bone marrow biopsy sections.

There is no information in the literature regarding the lymphocyte content or type in bone marrow biopsies from patients with "idiopathic" pure red cell aplasia (PRCA). This report describes the bone marrow biopsy sections of a patient with PRCA. A diffuse CD3 positive (CD8 positive, granzyme B negative) lymphocytosis of approximately 1500/mm3 was revealed by immunohistochemical staining. The extent of the T cell increase was not evident from morphological examination of the bone marrow aspirate or biopsy, from flow cytometric analysis of the aspirate, or from the peripheral blood lymphocyte count. Therefore, immunohistochemical analysis should be performed routinely in this rare disease and the data acquired may help to inform the choice of treatment.

Novel and Mediterranean beta thalassemia mutations in the indigenous Northern Ireland population.

beta thalassemia is one of the most common genetic diseases worldwide resulting from aberrant beta-globin chain production. It is highly prevalent in regions with endemic malaria, but it is also present at low frequency in the indigenous populations of non-tropical areas such as Britain. Screening beta thalassemia trait individuals from Northern Ireland has detected 2 Mediterranean mutations, 39 (C --> T) and IVS-I-110 (G --> A); the previously reported IVS-II-850 (G --> A) mutation originally described in individuals of Scottish/English ancestry; and 2 novel mutations, initiation codon A --> C and 109 delG. Haplotype analysis indicates that the Mediterranean mutations are present on previously described haplotypes, suggesting that they have arisen due to migration. It remains to be established whether the novel mutations have arisen de novo in Northern Ireland.

Efficacy and response to intravenous anti-D immunoglobulin in chronic idiopathic thrombocytopenic purpura.

This review explored the effectiveness of anti-D in the management of chronic idiopathic thrombocytopenic purpura (ITP). Of 16 patients, 14 non-splenectomized and two splenectomized, with chronic ITP received anti-D immunoglobulin at a dose of 50-75 mcg/kg. A total number of 100 doses anti-D were given. Fourteen patients had previous treatment with steroids, which was discontinued either because of unresponsiveness or unacceptably high maintenance doses. Two patients had no previous treatments with any modality. Anti-D was given as a short i.v. infusion whenever platelet count dropped below 30 x 10(9)/l or patient was haemorrhagic or preoperatively. Response was defined as an absolute platelet count >30 x 10(9)/l or an increment by > or =20 x 10(9)/l. Response was obtained in 14 patients with a response rate of 87%. Fifteen patients were not on any other form of treatment at the time of anti-D therapy and one patient had a concurrent steroid therapy. The improvement in platelet count lasted for more than 8 weeks post-57% of anti-D infusions. We report two patients with previous splenectomy for ITP who responded to anti-D therapy. The side-effects profile was very mild with no patients required red cell transfusion.