Most multiple myeloma (MM) patients suffer from anaemia because of decreased production of erythrocytes. 1,2 Rarely autoimmune haemolytic anaemia (AIHA) complicates MM and the correlativity between AIHA and MM has not been clarified. [3][4][5] Here, we report one MM case complicated by Evans syndrome (AIHA complicated by thrombocytopenia).A 65-year-old woman was admitted with a 1-month fatigue and dizziness. On examination, she appeared pale and slightly jaundiced. Laboratory investigation showed: haemoglobin 36 g/L, red blood cell (RBC) 1.2 ¥ 10 12 /L, reticulocyte 16.69%, platelet 16 ¥ 10 9 /L; spherocytes, macrocytosis, polychromasia, immature granulocytes and normoblasts were seen in her peripheral blood; serum lactate dehydrogenase (LDH) 571.4 IU/L (109.0~245.0), haptoglobin 5 mg/L (20~190), indirect bilirubin 50.6 mmol/L and total bilirubin 16.3 mmol/L. Moreover, direct Coombs test for immunoglobulin G (IgG) was positive. Ham's test, sugar-water test and test for cryoglobulins were negative.At the same time, total serum protein 80.1 g/L, globulin 51 g/L and immunoglobulin A (IgA) 40.30 g/L, while IgG and immunoglobulin M were lower than normal criteria. Urinary kappa was 0.14 g/L (0~0.02), l < 0.05 g/L (0~0.05). Serum protein electrophoresis revealed M band. IgA kappa M-protein was 40.20 g/L. On bone marrow aspiration: hyperplasia, erythrocytic series 42% and plasma cells occupied 15%, which were round or oval; some of them were binuclear, and some contained nucleoli; megakaryocytes were normal; on biopsy, a nodular infiltration of plasma cells was observed. Renal function, serum calcium and bone marrow chromosome were normal. Skeleton survey revealed no abnormalities. Ultrasound found splenomegaly.With flow cytometer technology, the subtype of the IgG binding to the erythrocytes was identified: it was IgG1. Her bone marrow mononuclear cells were cultured in serum-free medium for 72 h. Then the supernatant was harvested. IgA kappa in the supernatant was confirmed, while IgG did not exist. And the supernatant could not reduce erythrocytes from a volunteer with the same blood type to agglutinate.Evans syndrome was diagnosed. Five units of washed red cells, which did not cross-react with the patient's serum, were transfused. Moreover, 50 mg/day prednisolone with a reduction of 5 mg/week and 100 g human immunoglobulin were administered.Seven weeks later, restaging showed: haemoglobin 113 g/L, RBC 3.4 ¥ 10 12 /L, reticulocyte 0.42%, platelet 68 ¥ 10 9 /L; morphology of erythrocytes was normal, no immature granulocytes and normoblasts in peripheral blood. Her serum LDH and bilirubin were normal, while direct Coombs test remained slightly positive. Her serum IgA was 34.40 g/L; IgA kappa M-protein was 34.20 g/L. On bone marrow aspiration, erythrocytic series 20%, while plasma cells 20.8% and they mimicked the plasma cells 2 months ago. Ultrasound showed spleen was normal. Thus smouldering MM was confirmed.In this patient, IgG was responsible for AIHA, while the IgA kappa M-protein derived from the myeloma cells was...
