444 Correspondence MDS or AL secondary to chemoradiotherapy for malignancy ( 1 9 patients) or rheumatoid arthritis (one patient). Three patients with end-stage, therapy-resistant disease were excluded from this analysis. Since no patient had therapy- linked refractory anaemia (RA) at the time of BMT, only data of patients with AL, refractory anaemia with excess of blasts in transformation (RAEBt) or RAEB were analysed. Six out of the 17 patients with therapy-linked MDS or AL died from transplant-related complications, compared to 1 3 out of 45 patients without previous cytotoxic therapy ( P > 0.5). Also the incidence of GVHD and interstitial pneumonia was not significantly increased. No spontaneous report was made of GVHD restricted to previously exposed areas, but this infor- mation was not specifically sought for.

The second major issue of Dr Bandini’s letter is whether a patient with therapy-linked AL or MDS should be induced into complete remission (CR) prior to BMT or not. Ten out of 20 therapy-linked MDS or AL (AL 3 , KAEBt: 4, RAEB: 3 ) have not received chemotherapy with the option to induce CR. Three patients relapsed, three died from transplant- related complications, and four are alive without evidence of disease at 7 2 + , 59+, 56’ and 1.5+ months after BMT. Only one patient transplanted with overt untreated AL is alive without evidence of leukaemia for more than a year. This patient was transplanted with a hypocellular marrow and she is one of the cases mentioned by Dr Bandini (Marmont & Tura, 1986). Seven patients received a transplant early after initiation of chemotherapy (five AML in CR and two RAEB with therapy-induced hypoplasia). None of these patients

relapsed: four are alive without evidence of disease at 6 + , 44+, 50+ and 91 + months after BMT and three patients died from transplant-related complications. Three patients with therapy-resistant AML died from interstitial pneumoni- tis.

The numbers are small, but patients transplanted for overt therapy-linked AL fared less well after BMT compared to patients transplanted in CK, similar to the results observed in the overall population transplanted for MDS or s-AML. More data and more follow-up will be needed to substantiate these observations. At present, data from 119 patients trans- planted for MDS or secondary AL have been collected and future analyses will be directed at these issues.

Department ofInterrial Medicine T. DE WITTE University Hospital Nijniegen, The Netherlands Leukaemia Working Party

on behalf of the EBMTG

REFERENCES

De Witte, T.. Zwaan, F.. Hermans, J., Vernant. J.. Kolb, H.. Vossen. J.. Lonnquist, B., Beelen. D.. Ferrant. A,. Gmur. J.. Liu Yin, J.. Troussard. X.. Cahn. J., Van Lint. M. & Gratwohl, A. (1 990) Allogeneic bone marrow transplantation for secondary leukaemia and myelodysplastic syndrome: a survey by the Leukaemia Working Party of the European Bone Marrow Transplantation Group (EBMTG). British Jourriul of Huernatology, 74, 151-1 55.

Marmont. A.M. & Tura, S. (1986) Bone marrow transplantation for secondary leukaemia. Report of two cases. Bone Murrow Trunsplun- tation, i, (Suppl. 1). 191-193.

PRODROMAL CUTANEOUS VASCULITIS IN MYELODYSPLASTIC SYNDROMES

We read with interest the recent report by Green et aZ(1990) concerning the concomitant finding of cutaneous vasculitis and myelodysplastic syndromes (MDS) in elderly patients. We

would like to report four young patients with MDS in whom vasculitis was the presenting feature. Clinical and haemato- logical features at presentation to hospital are summarized in

Table I. Clinical and haematological data

Time to Age Presenting diagnosis Hb MCV WBC Neutrophils Monocytes Platelets ESR

Case (yr) Sex skin feature of MDS (g/dl) (fl) ( x lOY/l) ( x 10’11) ( x lo9/]) ( x loy/]) (mm/h) Marrow Chromosomes

1* 43 M Vasculitis. 3years 9.6 116 6.2 4.5 0.3 95 107 RA 46XY lower legs

2 25 F Rashon face 6 weeks 9.6 109 6 .7 1.6 0.4 231 85 RA/RAEB Complex buttocks and inc. monosomy legs 5 and 7

3 29 F Orogenital 3 weeks 5.6 107 2.5 0.9 0.1 52 48 RAEB-t 46XX ulceration erythema nodosum

4 31 M Pyoderma 4weeks 10.0 118 41.1 26.7 5.8 433 56 CMML 46XY gangrenosum

RA = refractory anaemia, RAEB( - t) =refractory anaemia with an excess of blasts (in transformation). CMML = chronic myelomonocytic leukaemia. * Antinuclear factor was weakly positive at 1 : 40 although no other evidence of a connective tissue disorder could be found. Autoantibodies.

rheumatoid factor, direct antiglobulin test and serum immunoglobulins were normal in all the cases. Lupus anticoagulant, anticardiolipin antibodies and cryoglobulins were normal in cases 1-3 but were not done in case 4.

Correspondence 445

Fig l(a). Pyoderma gangrenosum of the lower abdomen in case 4. There is a central ulcer with a necrotic purulent base and an elevated bluish-red border.

Fig I(b). Small dermal blood vessel showing fibrinoid necrosis of its wall and occlusion by thrombus. Lymphohistiocytic infiltration is also present. (H & E. x 560.)

Table I. Pyoderma gangrenosum seen in case 4 is shown in Fig l (a ) . Fig I (b) shows the histological features of the skin biopsy in case 2.

All four of our patients initially presented to the dermatolo- gists. Prodromal cutaneous vasculitis antedated the diagno- sis of myelodysplasia by a variable interval in all four cases (see Table I). In cases 1 and 4 high dosage steroids were required to control vasculitis. Case 2 showed little improve- ment with steroids. Improvement of the vasculitis accompa- nying chemotherapy was seen in cases 3 and 4, although this has not been reported to be a consistent feature (Greer et aI. 1988). Improvement in the severity of the vasculitis as the

bone marrow function deteriorates has been reported (Greer et al, 1988), although this was not observed in one of our cases (case 2 ) whose disease transformed from refractory anaemia to refractory anaemia with an excess of blasts.

Vasculitis is well recognized amongst both lymphoprolifer- ative and myeloproliferative disorders (Greer et al. 1988): however, only a few cases have previously been reported in association with myelodysplastic syndromes (Jacobs et al. 1985: Marti et al. 1987; Doutre et al, 1987). The exact mechanism of vasculitis in these cases remains unclear. MDS are known to involve a defect of a common lymphoid myeloid stem cell (Janssen et al, 1989) and are associated with

446 Correspondence peripheral blood lymphopenia involving B, NK and T cells of both helper/inducer (CD4) and cytotoxic/suppressor (CD8) subsets (Ayanlar-Batuman et al, 198 7) . These observations raise the possibility of altered immune surveillance which may lead to aberrant host reaction to tumour antigens identical to those of classical hypersensitivity vasculitis.

Departments of Haematological Medicine, *Dermatology and t Histopat hology, King’s College Hospital School of Medicine

and $Department of Haeinatology, Charing Cross Hospital. London

A. PAGLIUCA E. HIGGINS* D. SAMSON$

G. J. MUFTI and Dentistry, London, s. HdMPHRIESt

KEFERENCES

Ayanlar-Batman. 0.. Shevitz. 1.. Traub. tJ.C., Murphy, S. & Sajewski. D. (1987) Lymphocyte interleukin 2 production and responsive- ness are altered in patients with primary myelodysplastic syn- drome. Blood. 70, 494-500.

Doutre. M.S., Beylot. C.. Beylot. J., Courouge-Dorcier. D., Reiffers, I.,

Broustet. A. & Bioulac-Sage, P. (1 98 7) Refractory anemia with an excess of blasts and cutaneous vasculitis. Annals ofDermatology and Venereology, 114, 97-100.

Green. A.R.. Shuttleworth, D.. Bowen. D.T. & Bentley. D.P. (1990) Cutaneous vasculitis in patients with myelodysplasia. British Journal of Haernatology, 74, 364-365.

Greer. J.M., Longley, S., Edwards. L.N., Elfenbein, G.J. & Panush, R.S. (1988) Vasculitis associated with malignancy. Experience with 13 patients and literature review. Medicine (Baltimore). 67,220-230.

Jacobs. P.. Palmer. S. & Gordon-Smith, E.C. (1985) Pyoderma gangrenosum in myelodysplasia and acute leukaemia. Postgra- duate Medical Journal, 61, 689-694.

Janssen. J.W.G., Buschle. M., Layton. M.. Drexler, H.G.. Lyons, J.. van den Berghe, H., Heimpel. H., Kubanek, B., Kleihauer. E., Mufti. C.J. & Bartram, C.R. (1989) Clonal analysis of myelodysplastic syn- dromes: Evidence of multipotent stem cell origin. Blood. 7 3 ,

Marti. J.M., Cervantes. F.. Ribera. J.M.. Martin-Ortega. E. & Rozman, C. ( 198 7) Polyarthritis, cutaneous vasculitis and migrant throm- bophlebitis of possible immune origin associated with chronic myelomonocytic leukemia. Sangre, 32, 502-505.

248-254.