Occurrence Of Langerhans Cell Histiocytosis Research Articles

Recalcitrant Pruritus as Primary Manifestation of Synchronous Hodgkins Lymphoma and Langerhans Cell Histiocytosis

We present a 73 year old female with intractable pruritus and nonspecific cutaneous rash for a period of 9 months. She had recieved symptomatic therapy with no improvement. A complete examination revealed axillary and abdominal lymphadenopathy. A biopsy confirmed the diagnosis of Hodgkins lymphoma with Langerhans cell histiocytosis. She received 5 cycles of chemotherapy with resolution of pruritus and reduction in axillary and abdominal lymphadenopathy. The patient presented 6 months later with relapse and succumbed to the illness. Simultaneous occurrence of Langerhans cell histiocytosis and Hodgkins lymphoma may lead to misdiagnosis. The awareness of such an association is important to make an accurate diagnosis and guide appropriate therapy.

Multisystem langerhans cell histiocytosis in adult

Langerhans cell histiocytosis (LCH), is a rare disorder, clinically presents with heterogeneous manifestations, and has an unpredictable outcome. Commonly seen in infancy or early childhood, the disorder is characterized by proliferation of abnormal and clonal Langerhans cell in skin, bone, lymph nodes, lungs, liver, spleen, and bone marrow. Occurrence of LCH in adults is rare. Here, we report the case of an adult with acute onset of polymorphic eruptions all over the body, which on biopsy showed features of multisystem LCH, and was confirmed by immunohistochemistry. Although multisystem LCH has a poor prognosis, our patient responded well to chemotherapy.

Common Acute Lymphoblastic Leukemia Ph＋ Following Langerhans Cell Histiocytosis in a Multi-Malformed Child with INV (9) (p12;q13) (mat):Case Report

The occurrence of Langerhans cell histiocytosis (LCH) and another malignancy in the same patient is infrequent but has been recognized. The genetic changes that could be responsible for LCH and/or concomitant leukemia development are obscure. To the best of our knowledge, this is the first description of constitutional maternally derived inv (9) (p12;q13) in an LCH patient, and also of the development of common ALL Ph after LCH diagnosis and therapy. The potential significance of these findings [inv (9)+LCH+ALL Ph+] and their mutual relationship are unknown. Therefore, cooperative studies of large numbers of patients are needed to identify the common risk factors, if any.

Cholesteatoma Secondary to Temporal Bone Involvement by Langerhans Cell Histiocytosis

To describe secondary acquired cholesteatoma in patients previously treated for Langerhans cell histiocytosis (LCH). To focus on misleading symptoms suggesting LCH relapse. This study involved 3 patients aged 12, 20, and 58 months whose conditions were diagnosed with LCH. All 3 had involvement of the mastoid and of the skin of the external ear canal (EAC) at diagnosis. They were treated with steroids and vinblastine. Serial computed tomographic (CT) scans and clinical follow-up. Exploratory surgery of the mastoid. Otorrhea recurred in all 3 patients at 24, 17, and 26 months, respectively, with difficulties to clinically identify a hole in the posterior part of the EAC. The otorrhea was considered a sign of a new occurrence of LCH, leading to systemic chemotherapy in 2 cases. A CT scan showed a defect in the posterior wall of the EAC and suggested cholesteatomatous invasion of the mastoid (2 true cholesteatomas and 1 precholesteatomatous case).Surgery (canal wall up mastoidectomies) successfully removed the cholesteatoma (bilateral in 2 cases) and reconstructed the bony defect using cartilage. Biopsies ruled out LCH recurrence. Secondary acquired cholesteatoma (through a bony defect of the EAC) may occur in patients previously treated for LCH. Recurrence of symptoms and bone destruction on CT may wrongly suggest LCH recurrence. Surgery allows removal of the cholesteatoma as well as EAC reconstruction and control biopsy.

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

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. (J Am Acad Dermatol 2001;45:S233-4.)

Langerhans' Cell Histiocytosis on the Vulva

Background. Langerhans' cell histiocytosis (LCH) affecting the vulva alone is rare. At present, there are 51 published cases of LCH affecting the genital area. Of these, only 7 had LCH located in the genital area alone; in these cases, there was no subsequent systemic spread of the disease. A report of the 8th such case is presented with a review of the previous 7 cases.Case. A 40-year-old white woman presented with a several-month history of pruritic and ulcerous lesions on the vagina which had then spread to the vulva. No tiredness, general malaise, or associated temperature was observed. Physical examination disclosed an erythematous plaque on the labia minora, which was bilateral and slightly infiltrated. The rest of the physical examination brought no other disorders of interest to light. Histological findings were characteristic of LCH. Four cycles of iv vincristine (2 mg/m cycle) 15 days apart did not obtain objective or symptomatic improvement in the vulvar lesions. Therefore, a local and partial extirpation of both labia minora was performed. Eighteen months after surgery, the patient has no symptoms or signs of local recurrence or systemic spread.Conclusions. Although the occurrence of LCH on the vulva is very unusual, we must bear this possibility in mind when a woman presents atypical chronic lesions on the genital mucosa. In such cases, it is necessary to perform a biopsy on the mucosa, rule out the possibility of systemic disease, and review the patient periodically in order to forestall a possible spread of the disease at any time.

OCCURRENCE OF ACUTE NONLYMPHOBLASTIC LEUKEMIA IN TWO GIRLS AFTER TREATMENT OF RECURRENT, DISSEMINATED LANGERHANS CELL HISTIOCYTOSIS

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.