Abstracts : Langerhans histiocytosis and Leukemia

Ann Dermatol Venereol. 1998 Feb;125(2):124-6.

[Article in French]
Hermanns-Lê T, Arrese JE, Piérard GE.
Service de Dermatopathologie, Université de Liège.
INTRODUCTION: Cutaneous manifestations of myelodysplastic syndromes are rare and polymorphous. They can be the direct expression of the hematological disease or represent signs of a vasculitis or a neutrophilic syndrome. Myelodysplastic syndromes progress sometimes toward an acute leukemia. OBSERVATION: A 53-year-old woman suffering from myelodysplastic syndrome presented for several months a cutaneous vasculitis without any histological specificity. In time, such presentation was complicated by the simultaneous occurrence of a cutaneous Langerhans cell histiocytosis and an acute monoblastic leukemia type LMA 4. The disease was rapidly fatal. DISCUSSION: The complication of a myelodysplastic syndrome by concurrent Langerhans cell histiocytosis and acute monoblastic leukemia suggests a pathogenic relationship between these two latter disorders.

Egeler RM, Neglia JP, Aricò M, Favara BE, Heitger A, Nesbit ME.
Sophia Children's Hospital, Rotterdam, The Netherlands.
Langerhans cell histiocytosis (LCH) and malignancy occurring in the same individual is unusual and has generally been the subject of isolated case reports. To better define the occurrence of these events a registry of cases with synchronous or asynchronous LCH and malignancy was developed with the cooperation of the Histiocyte Society. In 1991 the Histiocyte Society surveyed its members requesting information on cases in which LCH was associated with malignancy. The questionnaire was mailed to all members of the society and specifically requested information on the clinical and laboratory features of the cases, disease evolution, and response to therapy. Retrospective reporting was allowed. With this initial data, an ongoing registry of LCH patients with associated malignancy was begun of such cases, including evolution and response to therapy. Twenty-seven patients were enrolled during the first year of registry, of whom 4 patients had the association of LCH with a malignant lymphoma and 10 cases had an association of LCH with other types of solid tumor. The remaining 13 patients had the association of LCH with acute leukemia. In five cases, LCH was associated with acute lymphoblastic leukemia FAB L1 (ALL). In four cases the ALL preceded the LCH by 6 months to 1 year. In four of five patients the LCH was localized; in two instances the LCH was treated with chemotherapy. In all cases the leukemia was treated according to local standard ALL protocols and in one case autologous bone marrow transplantation (ABMT) was performed at relapse. Three patients are free of leukemia, one of whom has persistent localized LCH of the skin. Two patients died of the ALL, one of whom was free of the LCH at the time of death. In eight instances LCH was reported in association with acute myeloid leukemia (AML). Six of these patients had a generalized form of LCH. In seven the diagnosis of LCH preceded the diagnosis of leukemia by more than 2 years (median 4 years). In the remaining patient both diagnoses were made concurrently. In all seven cases in whom LCH was the initial diagnosis the treatment consisted of chemotherapy and/or radiotherapy. Seven patients died from the AML, five without evidence of LCH. The temporal patterns of the LCH-ALL and LCH-AML associations are distinct with ALL usually preceding the diagnosis of LCH and AML succeeding it.(ABSTRACT TRUNCATED AT 400 WORDS)
PMID: 8202046 [PubMed - indexed for MEDLINE]

Aricò M, Comelli A, Bossi G, Raiteri E, Piombo M, Egeler RM.
Department of Pediatrics, University of Pavia, IRCCS Policlinico S. Matteo, Italy.
Langerhans cell histiocytosis (LCH) is a non-malignant disorder, whether localized or disseminated, and usually has a favourable prognosis. A possible relationship between LCH and neoplastic diseases has not been assessed up to now even if a few cases have been recorded. We report two new cases of acute leukemia in children with LCH. The first child had acute lymphoblastic leukemia after untreated LCH; the second developed acute promyelocytic leukemia after LCH treated with vinblastine and etoposide. To our knowledge, this is the first case of secondary leukemia after exposure to an epipodophyllotoxin derivative in a child with benign disease. Cooperative studies of large numbers of LCH patients are needed to evaluate a possible association between LCH and acute leukemia, and to identify common risk factors or predisposing agents if such be present.

[Article in Japanese]
Okubo T, Asou N, Suzushima H, Matsumi S, Uchiba M, Okuno Y, Nishimura S, Takatsuki K, Kawano F.
Second Department of Internal Medicine, Kumamoto University Medical School.
Six of 14 patients with acute myeloblastic leukemia (AML) complicated reactive histiocytosis during initial remission induction therapy. All six patients had a high fever without signs of infection during initial chemotherapy, and periods of myelosuppression were prolonged. Histiocytes with a mature appearance, some of which phagocyted erythrocytes, thrombocytes or neutrophils, increased in the bone marrow. All of 3 patients tested showed high serum levels of ferritin. Two of 3 patients treated with 125 mg/day methylprednisolone achieved complete remission. In the remaining 3 patients, one patient achieved complete remission, but the others died of fungal pneumonia or sepsis. Thus, reactive histiocytosis is one of the severe complications in patients with AML undergoing chemotherapy.

Segal GH, Mesa MV, Fishleder AJ, Stoler MH, Weick JK, Lichtin AE, Tubbs RR.
Department of Pathology, Cleveland Clinic Foundation, Ohio.
BACKGROUND. Langerhans cell precursors are considered to be identical to their mature counterparts except for the lack of Birbeck granules. Proliferations composed of such histiocytes appear to be uncommon. METHODS. Standard immunophenotypic, molecular genetic, and DNA content studies were used to characterize various hematopoietic disorders, including a proliferation of precursor Langerhans cells, which arose sequentially in a patient. RESULTS. The patient studied initially had a low-grade, B-cell, non-Hodgkin lymphoma and subsequently had an unusual histiocytic proliferation (precursor Langerhans cell histiocytosis) in cutaneous and lymph node sites. The patient eventually died of acute myelogenous leukemia (FAB, M5). CONCLUSIONS. A larger series is required to determine the significance of the precursor Langerhans cell phenotype, particularly with respect to the development of acute myelogenous leukemia.

Miano C, Bosman C, Boldrini R, DonFrancesco A, Ceci A, Mancini S, Rosati D.
Divisione di Ematologia Pediatrica, Ospedale Bambino Gesù, Roma, Italy.
The authors report on a case of disseminated Langerhans' cell histiocytosis with a clinical presentation and a bone marrow simulating, at onset, an acute leukemia non lymphoid. A hepatic needle biopsy performed for the progressive enlargement of the liver oriented the diagnosis towards a Langerhans' cell histiocytosis. The morphological, immunohistochemical and ultrastructural study of these cells showed them to be undetermined, i.e. Langerhans' cell precursors.

Fontana J, Koss W, McDaniel D, Jenkins J 3rd, Welton W.
The concept of a mononuclear phagocytic system consisting of a continuum of cells arising from the bone marrow monoblast and terminating in the mature tissue macrophage is being actively investigated. The presentation of documented acute monocytic leukemia 18 months following the confirmed diagnosis of histiocytosis X in a 39-year-old man strongly supports the concept of the mononuclear phagocytic system lineage.