HAEMOPOIETIC RECOVERY FROM Ph1 POSITIVE CHRONIC GRANULOCYTIC LEUKAEMIA

appears to have shown evidence of autoimmune haemolytic anaemia (AIHA). an important and well-recognized complication of other lymphoproliferative disorders. However. on reviewing the literature we have been unable to find a report of direct antiglobulin test (DAGT) positive haemolysis in HCL since the original description of leukaemic reticuloendotheliosis by Bouroncle et al (1958). We wish to draw attention to a recent case of lifethreatening haemolysis of very unusual antibody specificity in a case of HCL which proved refractory to all measures except intensive plasma exchange. A 66-year-old female presented in 1982 with a 2 months history of lethargy. weight loss and abdominal discomfort. There was marked pallor and splenomegaly. A diagnosis of HCL was made on the basis of typical ‘hairy’ cells in the peripheral blood, showing acid phosphatase positivity with tartrate resistance (TRAP), characteristic transmission electron microscopy (EM) features, and a diffuse lymphoid infiltrate on trephine bone biopsy with a prominent increase in reticulin. At diagnosis there was evidence of mild haemolysis with reticulocytes 4.8% and serum bilirubin 30 pnol/l (normal 317). Haemoglobin (Hb) was 5.8 g/dl. The DAGT was strongly positive with IgG coating (subclass IgGl). Free antibody was not detectable in the serum but an eluate showed anti-en’ specificity, subtype FS. Antinuclear factor (AM) was negative. Red cell transfusions were given without problems before splenectomy (splenic weight 3 kg). The patient maintained her Hb for 7 d when a haemolytic crisis occurred with free autoantibody in the serum (same specificity). Schumms test for methaemalbumin was positive and Dlasma Hb was increased. Profound intravascular antibody level from a titre of 16 to undetectable levels. The patient remains perfectly well on no therapy 4 years later. The DAGT is now negative. None of the other features of autoimmune disease mentioned by Westbrook & Golde (1985) were evident in this case. AIHA was life-threatening and very difficult to manage in this patient. The only previously reported cases (Bouroncle e t al, 1958) showed resolution of haemolysis post-splenectomy. We report this case to stress that AIHA. although rare, should be regarded as an important autoimmune complication of HCL. In addition the antibody (anti-Ena) was of unusual specificity for AIHA (Issitt et al, 1976; Pavone et al. 198 1 ). Plasma exchange was embarked upon only as a last resort as it has been regarded as of no benefit in warm AIHA (Mollison. 1979). In this instance it proved to be life saving and may merit further trial in cases of resistant AIHA.