Peter W. G. Saunders

Clinically significant newly presenting autoimmune thrombocytopenic purpura in adults: a prospective study of a population-based cohort of 245 patients.

Summary. The true incidence and prognosis of autoimmune thrombocytopenic purpura (ITP) in adults is unknown. We present the results of a prospective study in a population‐based cohort of newly presenting adults (≥ 16 years) with ITP and platelet count of < 50 × 109/l, which took place between 1 January 1993 and 31 December 1999 in the former Northern Health Region in the UK (population 3·08 million). A total of 245 cases were confirmed by bone marrow examination with a median follow‐up of 60 months (range 6–78 months). There were 134 females/111 males (1·2:1). Overall incidence was 1·6 per 105 per annum. Absolute incidence was similar for both sexes, with highest age‐specific incidence in those aged > 60 years. Thirty patients (12%) presented with frank bleeding, and 28% were asymptomatic. Forty‐five patients (18%) received no treatment, and 135 (55%) received first‐line treatment only. Thirty patients (12%) underwent splenectomy. There were four deaths (1·6%) from bleeding and/or the complications of therapy in this cohort, but only one was in the acute phase of the illness. The majority of patients (155 out of 245) achieved remission (platelet count > 100 × 109/l), with a further 59 (24%) in partial remission with no symptoms (platelet count 30–100 × 109/l). This population‐based study suggests that the traditional view of adult ITP as being a predominantly chronic disease that preferentially affects females needs to be modified.

HUMAN PARVOVIRUS INDUCED CYTOPENIAS: A REPORT OF FIVE CASES

12 1 patients of the Myeloma Group of Central Sweden. They are in doubt, however, as to the clinical significance and importance of correcting the s-pzm for creatinine. In the MRC series, of 476 patients, the uncorrected values were more predictive than corrected ones (Cuzick et ul, 1985). Alexanian et a1 (1985) in their analysis of data from 97 patients also recently concluded that there was nothing to be gained from adopting a correction formula. This accords with our own experience. Obviously, impaired renal function can make an appreciable contribution to the increases in s-p2m in myelomatosis. This does not diminish its impressive prognostic predictive power.

Northern region asplenia register--analysis of first two years.

OBJECTIVES: To assess the feasibility of setting up a register of patients with asplenia within a defined geographical area; to ensure that guidelines on best practice were implemented; to obtain information on antibody levels to pneumococcal capsular polysaccharides and Haemophilus influenzae type b capsular polysaccharide, before and after immunisation and annually thereafter; to raise awareness of risks among clinicians and to offer advice on management. DESIGN: Prospective recruitment using multiple sources of recruitment. Annual follow up reminders sent from Registration Centre. SUBJECTS: Population of (old, pre-1995) Northern Health Region: approximately 3.1 million. MAIN OUTCOME MEASURES: Data were obtained on reasons for asplenia, duration of asplenia, use of prophylactic antibiotics, Medic-Alert bracelets, immunisations, antibody levels, death. RESULTS: The register was initiated at the beginning of April 1995 and ran to the end of March 1997. After two years of operation, 1111 cases had been registered but the response from some health districts was poor. Major primary causes of asplenia were trauma (264), other surgical (198), lymphoproliferative disease (154), and idiopathic thrombocytopenic purpura (147). There were 664 patients on prophylactic antibiotics, of whom 498 were on continuous antibiotics. Only 18 had any type of warning bracelet. Antibody measurements were carried out at least once on 75% of patients; 306 patients had satisfactory antibody levels on first blood sample in year 1, rising to 405 in year 2; 43 patients failed to make any antibody response to Pneumovax despite multiple immunisations, and three patients failed to respond to Hib vaccine. Sixteen patients with satisfactory antibody levels in year 1 had low levels in year 2 requiring vaccine boosters. Sixteen deaths were reported, two of which were directly attributable to overwhelming sepsis. CONCLUSIONS: Registration has been successful and has raised awareness of the management of asplenia. Compliance with antibiotic prophylaxis and immunisation was initially poor. A potential high risk group of vaccine non-responders has been identified and poor persistence of pneumococcal antibodies has been identified which is likely to alter approaches to immunisation in asplenic patients.

Quantification improves the prognostic value of CD38 expression in B-cell chronic lymphocytic leukaemia

Summary. Recent studies have shown that CD38 expressed as a percentage of the antigen positivity can predict prognosis and disease progression in patients with B‐cell chronic lymphocytic leukaemia (B‐CLL). The present study showed that quantification of CD38 expressed as antibody‐binding capacity (ABC) improves the prognostic value of the percentage of CD38 positivity in B‐CLL. In a cohort of 81 patients with B‐CLL, a level of CD38 expression of ≥ 30% and an ABC value of 250 proved statistically valid cut‐off points to predict disease progression (% CD38: P=0·0027; ABC: P < 0·0001). There was a positive and significant correlation between the percentage of CD38 expression and ABC (r=0·7; P < 0·0001). There was a better discrimination of survival using ABC rather than percentage CD38 positivity (P < 0·0001 compared with P=0·0027). Only ABC predicted for survival in patients under 60 years of age (P=0·0076) or with stage A disease (P=0·0195). Both percentage CD38 and ABC discriminated between time to first treatment for all patients but only ABC predicted time to treatment for stage A patients (P=0·0004). In conclusion, CD38 positivity is an important prognostic factor in B‐CLL. However, quantification of CD38 is superior to the percentage positivity and should be used clinically in conjunction with other variables of predictive value to identify B‐CLL patients that are likely to progress.

AUTOIMMUNE THROMBOCYTOPENIA IN NEOPLASTIC DISEASE

HAMMOUDA, F., QUAGLINO, D. & HAYHOE, F.G.J. (1964) Blastic crises of chronic granulocytic leukaemia. Cytochemical, cytogenetic and autoradiographic studies in four cases. British Medical Journal, i, 1275-12:77. HUNTER, J. & NELSON, M.G. ( I 966) Paroxysmal nocturnal haemoglobinuria. Zrish Journal of Medical Science, 485, 203-205. MAURI, C.. BALDUINI, C., QIJAGLINO. D., EMILIA, G., DAVOLI, P.G. & TRALDI, A. (1966) Indagini su alcuni aspetti metabolici e citogenetici dell’ emoglobinuria parossistica riotturna. Bollettino Societa Medico Chirurgica Modena, 66, 117. 191 1-19 12. ONI. S.B., OSUNKOYA, B.O. & LUZZATTO, L. (1970) Paroxysmal nocturnal hemoglobinuria: evidence for monoclonal origin of abnormal red cells. Blood, 36, 145-152. PARIS CONFERENCE (1972) Standardization in human cytogenetics. The National Foundation-March of Dimes. ROSE, W.F. (1972) In: Hematology (ed. by W. J. Williams, E. Beutler, A. J. Erslev and W. Rundles), p. 467. McGraw-Hill, New York. SANDBERG, A.A. (1980) The Chromosome in Human Cancer and Leukemia. Elsevier North Holland, New York. SEABRIGHT, M. (1971) A rapid banding technique for human chromosomes. Lancet, ii, 971-972. WHANG-PENG, J., KNUTSEN, T., LEE, E. & LEVENTHAL, B. (1 9 76) Acquired XO/XY clones in bone marrow of a patient with paroxysmal nocturnal hemoglobinuria (PNH). Blood, 47, 61 1619. ZACCARIA, A., ROSTI, G., BETTI, S., TESTONI, N. & BACCARANI. M. (1982) Normal karyotype in seven patients with paroxysmal nocturnal haemoglobinuria. British Journal of Haematology, 51, 333-334.

Response to Frederiksen and Schmidt

It was with great interest that we read the paper by Neylon et al (2003), which claimed to present for the first time a population-based cohort of adult idiopathic thrombocytopenic purpura (ITP) patients. However, we reported population-based incidence estimates of adult ITP in 1999, in a paper that, to the best of our knowledge, was unprecedented (Frederiksen & Schmidt, 1999). We provided incidence estimates for different age groups, for both sexes at different platelet count cut-off points, and across three different time periods. Using the same platelet count cut-off point as Neylon et al (2003), this gave us an estimated incidence in Danish adults at 3Æ2/100 000/year, twice the figure reported by Neylon et al (2003). Our incidence rates in all age groups were higher than those reported by Neylon et al (2003) in their prospective study, despite the fact that the platelet concentration distribution, number of asymptomatic patients, and median age of the cohorts (56 years) were very similar or identical. The reason for this discrepancy is not entirely clear. Our collection of data was retrospective but, in general, a prospective study would increase the awareness of a disease which would lead to an increased incidence if the cases were to be recruited solely from specialized departments. It is, however, not clear to us how the cases were recruited by Neylon et al (2003). In Denmark, all persons have a unique and permanent identification number [the Central Population Register (CPR) number], which is the key to individual information in all registries. The CPR covers the entire Danish population (from 1968 onwards), and the CPR number is a prerequisite for obtaining any form of social benefit, health care, education or salary (Frank, 2000). Identification of our ITP cases was facilitated by this CPR system. The higher incidence estimate in our study may be due to the fact that it is based on all ITP patients diagnosed in any hospital, any outpatient clinic, by private practising physicians, or general practitioners in the study region. Neylon et al (2003) did refer to our study, which was cited in support of an incidence of fatal haemorrhage in ITP reaching 10Æ4%. This statement is disconcordant with the (published) fact that only two (old) patients died from haemorrhage in our cohort of 221 patients.

Lymphoplasmacytoid lymphoma, associated paraproteinaemia and haemolytic anaemia: a report of 2 cases and review of the literature.

2 cases of lymphoplasmacytoid lymphoma are reported. Both were associated with a monoclonal paraprotein in serum and urine. In 1 case this was of the IgD subclass and is the first such case to be repo