Richter’s syndrome developing in a patient with adult onset Still’s disease

Abstract

Dear Editor, Autoimmune disorders can be a risk factor for the development of lymphoma [1], and at the same time, malignant lymphoma can present with paraneoplastic syndromes consisting of autoimmune phenomena and even full-blown rheumatoid disorders [2]. When a patient presents with predominant clinical symptoms of autoimmune disease awareness is necessary in order not to miss an underlying lymphoma. In this paper, we present a case that illustrates the difficulties encountered diagnosing malignant lymphoma when autoimmune phenomena dominate the clinical presentation. A 53-year old woman was referred to our hospital for a second opinion. Seven months earlier, she had been admitted elsewhere because of intermittent fevers, rigors, night sweats, anorexia, weight loss, myalgia, arthralgia, and a maculopapular rash. Before this, there was no relevant medical history. On physical examination, no pathological lymph nodes, splenomegaly, or hepatomegaly was present. Laboratory investigation revealed a leukocytosis (34.7× 10/l) with 90% neutrophils, elevated C-reactive protein (222 mg/l), liver test abnormalities [alkaline phosphatase (168 U/l), aspartate transaminase (76 U/l), alanine transaminase (49 U/l)], elevated lactate dehydrogenase (910 U/l), and hyperferritinemia (9,409 μmol/l). Additional laboratory tests and serology did not show signs of rheumatoid diseases, sarcoidosis, viral, or bacterial infections (e.g., Epstein–Barr virus (EBV), cytomegalovirus, parvovirus, human immunodefficiency virus, mycoplasma, and borrelia). Computer tomography (CT) revealed two small lymph nodes in the left axilla, but a biopsy failed to acquire representative material. Eventually, the diagnosis of adult onset Still’s disease (AOSD) was made because she met almost all criteria for this disease and no signs of a lymphoproliferative disease were found (Table 1) [3]. Treatment was started with prednisone (30 mg once daily), resulting in rapid clinical recovery. August 2006, the patient was referred because lymphocytosis (absolute lymphocyte count, 5.7×10/l) was noticed repeatedly during follow up, not accompanied by physical complaints or signs or cytopenias. After the prednisone was tapered and finally stopped, with immunophenotyping of peripheral blood (CD5, CD19, CD20, CD23, and Ig lambda positive), the diagnosis of a B-cell chronic lymphatic leukemia (B-CLL) could be established, with RAI stage 0, not necessitating therapy. A relation with the prior diagnosis of AOSD was not considered. In November 2006, she was readmitted because she experienced a recurrence of the same complaints earlier resulting in the diagnosis of AOSD. Again, leukocytosis (26.8×10/l) with predominantly neutrophils (90%) and 8% lymphocytes was seen. A skin biopsy revealed aspecific dermatitis compatible with AOSD. Because of high fever causing headaches, treatment with naproxen and paracetamol was initiated without benefit. Bone marrow biopsy confirmed the earlier diagnosed B-CLL showing small foci of lymphocytic B cells with expression of CD5 and CD23 (Fig. 1a,b). CT now revealed generalized lymphadenopathy Ann Hematol (2009) 88:81–84 DOI 10.1007/s00277-008-0526-4