Occurrence of Acute Nonlymphoblastic Leukemia in Two Girls After Treatment of Recurrent, Disseminated Langerhans Cell Histiocytosis

Abstract

The occurrence of Langerhans cell histiocytosis (LCH) and acute leukemia in one individual has rarely been observed. Despite few exceptions, two distinct patterns of association appear evident: acute lymphoblastic leukemia preceding LCH and LCH preceding acute nonlymphoblastic leukemia (ANLL). The latency of ANLL after the diagnosis of LCH is suggestive of a therapy-related process. This report describes two new cases in whom ANLL was diagnosed 7 years 8 months and 5 years 8 months after the start of initial treatment of disseminated recurrent LCH. Morphology showed blasts from FAB-type M4/M5 in the first patient, who died due to progression of leukemia. The second patient showed myelodysplastic syndrome (refractory anemia with excess of blasts in transformation; RAEB-t) and is now in remission from leukemia 3 years 11 months after allogeneic bone marrow transplantation. The review of a total of 26 patients with ANLL after LCH suggests that the disease has a poor prognosis and allogeneic BMT seems to be the treatment of choice.

Langerhans cell histiocytosis associated with myelodysplastic syndrome in adults

“…LCH has rarely been described in association with MDS in children 13–15 . One single case of LCH in association with MDS in an adult patient has been reported in the French literature 8 .…”

“…9,21,22 LCH has rarely been described in association with MDS in children. [13][14][15] One single case of LCH in association with MDS in an adult patient has been reported in the French literature. 8 To our knowledge, these two cases represent the first described in the English language literature and two of the only three cases described of LCH in association with MDS in adults.…”

“…Rarely, LCH may be associated with underlying diseases such as leukemia, lymphoma, and other solid malignancies 4,7–12 . Very rare cases of LCH have been described in children with myelodysplastic syndrome (MDS) 13–15 . We describe two cases of cutaneous LCH associated with MDS in adults.…”

“…4,7-12 Very rare cases of LCH have been described in children with myelodysplastic syndrome (MDS). [13][14][15] We describe two cases of cutaneous LCH associated with MDS in adults. To our knowledge these are the first such cases described in the English language literature.…”

Disseminated Langerhans cell histiocytosis associated with acute myeloid leukaemia: complete remission with daunorubicin and cytarabine

“…Concomitant LCH and AML have very rarely been reported [2,3]. Two explanations for this extraordinary phenomenon have been proposed: LCH and AML deriving from the same neoplastic precursors or LCH being reactive to the AML [1–3].…”