Ann Hematol (2008) 87:237–238 DOI 10.1007/s00277-007-0370-y
LETTER TO THE EDITOR
Acute lymphoblastic leukemia subsequent to autoimmune hemolytic anemia: a case report Pasquale Niscola & Laura Scaramucci & Alessio Perrotti & Stefano Fratoni & Daniela Piccioni & Andrea Tendas & Luca Cupelli & Teresa Dentamaro & Giovanni Del Poeta & Paolo de Fabritiis
Received: 16 June 2007 / Accepted: 12 August 2007 / Published online: 14 September 2007 # Springer-Verlag 2007
Dear Editor, Autoimmune hemolytic anemia (AIHA) is a serious complication of lymphoid malignancies, mostly chronic lymphocytic leukemia and B cell non-Hodgkin lymphomas [1, 2]. Very few descriptions of AIHA developed in patients affected by B cells acute lymphoblastic leukemia (ALL) [3, 4] have been reported. We recently observed and hereby report the case of a patient who developed an early pre-B ALL, massively infiltrating the ileum, 9 months after the diagnosis of AIHA, while the latter was in complete remission. In June 2006, a 56-year-old man came to visit referring severe malaise, fatigue, and the emission of hyperchromyc urine. On physical examination, he presented pallor, jaundice, and tachycardia; liver and spleen were palpable 2 cm below the costal margin. A comprehensive laboratory workup revealed a severe anemia associated with a typical Coombs-positive (warm antibodies) hemolytic pattern. A total-body computed tomography (CT) scan showed no pathologic findings. In particular, no signs of infiltration of the ileum were detected. Before starting steroids, a bone marrow (BM)
P. Niscola (*) : L. Scaramucci : A. Perrotti : D. Piccioni : A. Tendas : L. Cupelli : T. Dentamaro : G. Del Poeta : P. de Fabritiis Haematology Division, Sant’Eugenio Hospital, Tor Vergata University, Via dell’Umanesimo 10, 00144 Rome, Italy e-mail:
[email protected] S. Fratoni Pathology Department, Sant’Eugenio Hospital, Tor Vergata University, Rome, Italy
aspirate and a trephine biopsy were done; an erytroyd hyperplasia and a small amount of mature lymphocytes were observed. A high dose of prednisone was allowed for a rapid hematologic recovery, and he was discharged and regularly followed as an outpatient. Basing on hematologic findings, prednisone was progressively reduced and then definitively withdrawn 4 months later. In March 2007, he came to visit referring malaise and fatigue. A severe pancytopenia was found. A BM aspirate allowed the diagnosis of B ALL, L2 according to the French–American–British classification. The lymphoid blasts resulted as pre-B cells (CD10 and TdT positive) on immunophenotyping. The trephine biopsy taken at the time of the diagnosis of AIHA was retrospectively evaluated. No lymphoid cells expressing CD10 and/or TdT were present. The patient was enrolled in a clinical protocol and received a regimen including vincristine, daunorubicine, prednisone, and Lasparaginase as induction therapy. During aplasia, a septic shock because of Escherichia coli occurred and was then resolved by standard measures and broad-spectrum antibiotic therapy. The patient fully recovered, but 15 days later, a sudden bowel perforation complicated his clinical course; he underwent a laparotomy; a long tract of ileum, appearing necrotic, was removed. The patient’s clinical conditions progressively deteriorated until his death supervened for a septic shock refractory to all intensive care and resuscitating measures. On histological examination, the removed bowel tract showed a massive infiltration of Peyer’s plaques by immature lymphoid B cells and an extensive intestinal necrosis, involving all parietal structures. This case presents some peculiar features, such an AHIA followed by a pre-B cells ALL, which, at the best of our knowledge, has not been reported so far. A bidirectional association between autoimmune diseases and lymphoproliferative disorders has been recently
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claimed [2, 5]. In particular, a possible pathogenic link may be represented by the impairment of the immune surveillance and/or of the apoptotic mechanisms normally regulating the elimination of the autoreactive or transformed lymphoid cells [5]. Unfortunately, no biological studies to establish a pathogenic relationship between the two disease entities were available in our case, for which the question as to whether the occurrence of the two disorders was merely coincidental or represented different stages of a common underlying pathologic process remained unresolved. However, the close temporal relationship between the development of the AIHA and ALL, occurred as distinct disease entities, may suggest a possible pathogenic link, although the biological means of our observations should be elucidated by further descriptions of similar cases and by future research that are awaited in this setting.
Ann Hematol (2008) 87:237–238
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