Case Report Successful Treatment of Thrombocytopenia and Hemolytic Anemia with Ivig in a Patient with Lupus-Like Syndrome After Mismatched Related PBSCT

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Case Report Successful Treatment of Thrombocytopenia and Hemolytic Anemia with Ivig in a Patient with Lupus-Like Syndrome After Mismatched Related PBSCT Bone Marrow Transplantation (2001) 27, 337–340 2001 Nature Publishing Group All rights reserved 0268–3369/01 $15.00 www.nature.com/bmt Case report Successful treatment of thrombocytopenia and hemolytic anemia with IvIG in a patient with lupus-like syndrome after mismatched related PBSCT A Hartert, W Willenbacher, S Gu¨nzelmann, E Roemer, N Basara, AA Fauser and MG Kiehl Clinic of Bone Marrow Transplantation and Haematology/Oncology, Idar-Oberstein Germany Summary: weakness, edema, anemia, thrombocytopenia, and erosive cutaneous lesions. He had received a conditioning regimen Hematopoietic stem cell transplantation (HSCT) is a consisting of busulfan (4 mg/kg bw × day on days −7to treatment option for autoimmune diseases but can also −4) and cyclophosphamide (60 mg/kg bw × day on day −3 cause clinical features similar to those of autoimmune and day −2) before PBSCT. The donor was the patient’s diseases. In some of these cases the autoimmune-like sister. The patient’s blood group was A Rh positive, CMV- condition is associated with autoimmune cytopenia, a IgG seropositive and HLA-typing demonstrated HLA- complication that can be unresponsive to established A01/01, B 8/35, Cw 040/0701, DR 12/11, DRB1 120/110, treatment strategies and which may be fatal. The and DQB1 0301/0603. He received peripheral blood stem majority of cases reported on immune hemolytic anemia cells form his A Rh positive, CMV seronegative and HLA have been of alloimmune origin due to ABO red blood partially mismatched (DRB1 010×/110×; DQB1: cell antigen incompatibilities between donor and recipi- 0501/0603) sister. The graft contained 2.28 × 106 CD34+ ent. We now report a patient with a lupus-like syn- cells/kg/bw. drome, presenting with severe thrombocytopenia and GvHD prophylaxis consisted of cyclosporine (CYA) hemolytic anemia 9 months after HLA-mismatch, ABO starting on day −3, prednisolone starting on day +7 after compatible-related PBSCT who experienced no transplantation and MTX given as a short course on days response to high-dose steroids, but who had a sustained +1, +3 and +6 at a dose of 15 mg/m2 and twice at 10 mg/m2, response to repeated IvIG therapy. Bone Marrow Trans- respectively. Engraftment (leukocyte count Ͼ1000 × 109/l, plantation (2001) 27, 337–340. neutrophil count Ͼ0.5 × 109/l) occurred on day +21 after Keywords: autoimmune cytopenia; hematopoietic stem PBSCT. He developed slight cutaneous GVHD grade I–II cell transplantation; lupus-like syndrome; alloimmunity; which responded promptly to increased immunosuppres- IvIG sion with prednisolone (0.7 mg/kg bw × day) for 1 week, then tapered, and mycophenolate-mofetil (MMF) (2 g/day) for 3 months. CMV reactivation, as diagnosed by CMV-PCR, occurred Hematopoietic stem cell transplantation (HSCT) can cause 2 months after PBSCT (recipient CMV IgG positive, donor clinical symptoms characteristic of autoimmune diseases CMV IgG negative). This was successfully treated with like myasthenia gravis, immune thyroiditis, as well as ganciclovir, but the PCR became positive again shortly 1–11 immune cytopenia. Especially, autoimmune cytopenia is after cessation of therapy. He therefore received cidofovir 2,3 mostly refractory to standard therapeutic approaches. We as maintenance for 2 months. herein report a patient with a lupus-like syndrome associa- Immunosuppressive therapy on admission consisted of ted with hemolytic anemia and severe thrombocytopenia prednisolone 5 mg/day and CYA 150 mg/day given orally after HSCT. (blood level 284 ng/ml). Patient characteristics Clinical findings + Nine months (day 277) after a mismatched related allo- As papular cutaneous lesions were the main clinical finding geneic stem cell transplant (PBSCT) for CML in first CP, a viral infection was suspected, possibly herpes virus or a 38-year-old patient presented with weight gain, general VZV reactivation. CMV testing had been negative for the last 4 months. However, the CMV-PCR was positive at the time of admission. He was treated with foscarnet to avoid Correspondence: Dr MG Kiehl, Clinic of Bone Marrow Transplan- tation and Oncology/Oncology, Dr Ottmar-Kohler-Str. 2, 55743 Idar- further myelosuppression in a graft which was already Oberstein, Germany suboptimal. Testing for VZV and HSV was negative. Received 31 May 2000; accepted 4 October 2000 Within a few days of admission the patient developed Successful treatment of thrombocytopenia after PBSCT with IvIG A Hartert et al 338 fever, arthralgia, severe pancytopenia, generalized edema, resolved slowly. Colonoscopy demonstrated discrete polyserositis with pericardial and pleural effusions, and changes compatible with intestinal GVHD grade I. Thus, nephrotic syndrome. Creatinine levels increased to the prednisolone dose was increased from 5 mg/day to 176.8 ␮mol/l and creatinine clearance decreased to 12.5 mg/day, whereas the other immunosuppressive medi- 83 ml/min, with a mixed glomerular/tubular proteinuria of cation remained unchanged for the first days of inpatient 4.5 g/l. He had oliguria with a further weight gain of nearly treatment. Intestinal symptoms improved within 1 week. A 7 kg, and for 1 week he experienced diarrhea which com- few days after admission the patient developed a tempera- menced shortly after the onset of these symptoms. A bone ture up to 38.8°C. No infection focus was found. Testing marrow biopsy showed mild hypoplasia, incompatible with for other herpes viruses including VZV and EBV was nega- the severe peripheral cytopenia. Folic acid, vitamin B12 tive. With the onset of fever the hemoglobin and platelet and iron levels were within normal ranges; the ferritin level levels dropped to 4 g/dl and 6000/␮l, respectively, without was increased to 774 ng/ml. The hemoglobin decreased to increment after transfusion. Direct Coombs testing was 40 g/l, leukocyte count to 1.5 × 109/l from 3.1 × 109/l, and positive and blood smears showed fragmented cells due to platelet count to 6 × 109/l from 66 × 109/l (Figure 1). The hemolysis. The edema worsened with a further increase in reticulocyte count was 4.2%. Coagulation parameters were body weight up to 96.2 kg. Ultrasound and CT scan within the normal ranges. Endoscopy revealed no gastro- revealed a polyserositis. CYA medication for GVHD intestinal bleeding, but GVHD grade 2 of the bowel was prophylaxis was immediately stopped and replaced by diagnosed histologically. The pancytopenia was refractory MMF because of a possible CYA-induced microangiopa- to platelet and red cell transfusion. We observed a slight thy. Autoantibody screening was performed and findings increase in bilirubin levels to 27.36 ␮mol/l, but hapoglobins corresponded to an autoreactive process after allogeneic remained within the normal range (53 mg/dl). Blood smears PBSCT, comparable to a lupus-like syndrome that met six showed fragmented cells indicating severe haemolysis and of the ARC criteria for SLE (serositis, arthralgia, ANA, the direct Coombs test was positive. Further analysis Anti-ds-DNA, Coombs positive anemia, thrombocytopenia, revealed polyspecific antibodies and ANA titers which proteinuria, discoid skin lesions). We did not perform a increased from 1:20 000 to 1:40 000 within 3 weeks with kidney biopsy to assess the grade of renal involvement homogenous a IF-pattern, and concomitant slightly elevated because of the refractory thrombocytopenia. Neither patient anti ds-DNA antibodies (25 U/l and 30 U/l). Anti Scl 70, nor donor had a history of rheumatic disease. No other rela- Ro/SS-A, c-ANCA, p-ANCA, MPO, C3 nephritis factor, tive had suffered from autoimmune disease. anti-tubular and anti-glomerular basal membrane antibodies Treatment consisted of prednisolone 75 mg for 8 days, were negative. C3 complement was decreased to 68 mg/dl then reduced to 50 mg/day, and MMF 2 g/day. Intravenous (normal range 90–180 mg/dl) and C4 complement factor immunoglobulin (IvIG) treatment was started at a dose of was reduced to levels below 11 mg/dl (10–40 mg/dl). 0.4 mg/kg bw on 5 consecutive days. A total of four cycles Lupus-anticoagulant was negative. was given. Oliguria and edema were treated with furose- mide and xipermide. Renal function subsequently improved markedly. On day +329, 2 months after symptom onset, the Clinical course and therapy serum creatinine was within the normal range and pro- teinuria decreased from 4.5 g/l to 0.8 g/l. The patient’s body On admission (day +277 after PBSCT) skin rash, diarrhea weight declined from 96 kg (maximum) to his regular body three to four times a day, and pancytopenia were observed weight of 72 kg and he no longer required diuretics. Hemo- and interpreted as GVHD of the skin and bowel, associated globin level (50 g/l) and platelet count (23 × 109/l) were with graft dysfunction, possibly CMV associated. Joint still low at the time of discharge on day +368 and there pains were interpreted as symptoms of CMV disease. PCR were no clinical signs of anemia or bleeding. Hb and plate- testing was positive, but pp65 Ag was negative. Adminis- lets increased during the following 2 weeks to levels of tration of foscarnet, twice a day (90 mg/kg) improved the 70 g/l and 50 × 109/l, respectively. Three weeks later (day clinical symptoms of CMV disease and the cytopenia +398) the patient was readmitted with new self-limiting Platelet counts 140 120 IvIG /l) 9 100 80 60 40 Platelets (x10 20 0 270 275 279 283 291 297 305 335 341 370 400 421 447 533 590 751 Days post HSCT Figure 1 Response of platelet counts to IvIG treatment. ↓, IvIG treatment. Bone Marrow Transplantation Successful treatment of thrombocytopenia after PBSCT with IvIG A Hartert et al 339 episodes of arthralgia, decrease in platelet count Various drugs can induce an SLE-like syndrome. One (39 × 109/l) and Hb (37 g/l) associated with a slight of these is cyclosporine.
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