1: Pediatr Hematol Oncol. 1999 May-Jun;16(3):251-6. 

Comment in:
    Pediatr Hematol Oncol. 1999 May-Jun;16(3):183-5.

Occurrence of acute nonlymphoblastic leukemia in two girls after treatment of
recurrent, disseminated Langerhans cell histiocytosis.

Kager L, Heise A, Minkov M, Mobius D, Kotte W, Schulte-Overberg U, Henze G,
Gadner H.

St. Anna Children's Hospital, Vienna, Austria.

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.

Publication Types:
    Case Reports
    Review

PMID: 10326224 [PubMed - indexed for MEDLINE]

2: Med Pediatr Oncol. 1993;21(4):271-3. 

Langerhans cell histiocytosis and acute leukemia: unusual association in two
cases.

Arico 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.

Publication Types:
    Case Reports

PMID: 8469222 [PubMed - indexed for MEDLINE]

3: J Am Acad Dermatol. 2001 Dec;45(6 Suppl):S233-4. 

Langerhans cell histiocytosis in a child while in remission for acute
lymphocytic leukemia.

Chiles LR, Christian MM, McCoy DK, Hawkins HK, Yen AH, Raimer SS.

Department of Dermatology, The University Texas Medical Branch at Galveston,
77555-0783, USA.

The occurrence of Langerhans cell histiocytosis (LCH) and malignancy in the same
patient is rare. When LCH occurs concomitantly with acute leukemia, distinct
temporal patterns often exist; acute myelogenous leukemia (AML) typically
succeeds LCH, whereas acute lymphocytic leukemia (ALL) usually precedes it. We
report a case of LCH developing in a child while in remission for ALL. Unique
features of this case include the disseminated nature of the LCH and the death
of the patient from LCH rather than ALL.

Publication Types:
    Case Reports

PMID: 11712070 [PubMed - indexed for MEDLINE]

4: Am J Hematol. 2001 Dec;68(4):284-6. 

Langerhans cell histiocytosis following childhood acute lymphoblastic leukemia.

Raj A, Bendon R, Moriarty T, Suarez C, Bertolone S.

Department of Pediatric Hematology/Oncology, University of Louisville,
Louisville, Kentucky 40202, USA.

Langerhans cell histiocytosis (LCH) is a clonal proliferation of Langerhans
cells of unknown etiology that results in a range of clinical manifestations.
LCH has been known to be associated with a variety of malignant diseases. A
7-year-old boy was treated for standard-risk acute lymphoblastic leukemia (ALL)
at age 2 years, on a Children's Cancer Group chemotherapy protocol for 3 years
and developed LCH 2 years after completion of chemotherapy. The case and a
review of literature on the association of LCH and ALL are presented. Copyright
2001 Wiley-Liss, Inc.

Publication Types:
    Case Reports

PMID: 11754419 [PubMed - indexed for MEDLINE]

5: Leuk Lymphoma. 2005 Dec;46(12):1735-41. 

Histiocytosis following T-acute lymphoblastic leukemia: a BFM study.

Trebo MM, Attarbaschi A, Mann G, Minkov M, Kornmuller R, Gadner H.

St Anna Children's Hospital, Vienna, Austria. m.trebo@utanet.at

The coincidence of T-cell acute lymphoblastic leukemia (T-ALL) and histiocytic
disorders, including hemophagocytic lymphohistiocytosis (T-ALL/HLH) and
Langerhans cell histiocytosis (T-ALL/LCH), is very seldom and is usually
associated with a dismal prognosis. Retrospective statistical analysis of all
T-ALL patients, who have been registered in the BFM-ALL trials from 1981 - 2001
and who have subsequently developed a LCH/HLH, in order to identify any common
risk factors pre-disposing to the synchronous occurrence of both disorders. Six
out of 971 T-ALL patients had either HLH or LCH ( approximately 0.03% of treated
T-ALL/year). The mean age at diagnosis of T-ALL/HLH/LCH was significantly lower
than in the remaining T-ALL group (4.05 +/- 0.59 vs 8.82 +/- 0.14 years; p =
0.000). The mean initial leukocyte count was higher than in the non-HLH/LCH
group (270,700 +/- 60,677 microl(-1) vs 134,141 +/- 5,663 microl(-1); p =
0.074). No hemophagocytosis was seen in the initial bone marrow (BM) smears.
Five of 6 patients obtained a good prednisone response (GPR) at day 8 in
peripheral blood with <5% blasts at day 15 in BM and all cases were in complete
remission (CR) at day 33. The mean time until development of the histiocytosis
was 17.95 months (range 2.5 - 33 months). Four patients developed a HLH and 2 a
LCH. All patients with HLH showed a multi-organ involvement, while the LCH
patients had only local disease. Only the LCH patients survived, while all
patients with HLH died. The authors recommend a close follow-up for at least 3
years after diagnosis in younger T-ALL patients with high initial leukocyte
count.

Publication Types:
    Case Reports

PMID: 16263575 [PubMed - indexed for MEDLINE]