Adult-onset Langerhans cell histiocytosis presenting with adipsic diabetes insipidus, diabetes mellitus and hypopituitarism: A case report and review of literature

E, Mendoza; Lopez, Amy A.; Valdez, Valerie Ann U.; Uy-Ho, Jean D.; Kho, Sjoberg A.

doi:10.1016/j.jecr.2015.06.001

Cited by 5 publications

(9 citation statements)

References 17 publications

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“…Physicians should consider long‐term effects of the lesions in patients with progressive multiple PLCH. The structural changes in the hypothalamic–pituitary axis often herald involvement of other parts of the CNS and its attendant neurological sequelae, which may reflect the irreversible and permanent nature of this infiltrative disease [6]. Fortunately, our patient's visual ability was not affected by the anterior pituitary adenoma, which was located close to the optic chiasma.…”

Section: Discussionmentioning

confidence: 89%

“…The differential clinical classification of LCH is considered to include three stages: first, a single-system disease with good prognosis; second, a multi-system disease; and finally, multi-system disease with organ dysfunction and the worst prognosis [14,15]. In adults, LCH most often manifests as a multi-system disorder with particular involvement (5-50% of cases) of the pituitary gland in the hypothalamic-pituitary axis in the CNS [6,16,17]. More specifically, adult intracranial LCH cases involve infiltration of the posterior pituitary.…”

Section: Discussionmentioning

confidence: 99%

“…Pulmonary LCH (PLCH) predominantly affects young adult smokers between 20 and 40 years of age [5]. In general, PLCH is an interstitial lung disease; however, occasionally, clinical manifestations may include multi‐systemic dysfunctions like other types of LCH [6].…”

Section: Introductionmentioning

confidence: 99%

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Secondary pulmonary hypertension due to pulmonary Langerhans cell histiocytosis accompanied with panhypopituitarism

Miyashita

Yamazaki

Ohta

et al. 2020

Respirology Case Reports

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A 65-year-old man presented to our hospital with complaint of acute dyspnoea. He smoked 30 cigarettes per day from age 20 to 52 years. Immunocytochemical findings revealed 6.3% of positive CD1a cells in the cell fraction of bronchoalveolar lavage, thus suggesting a diagnosis of pulmonary Langerhans cell histiocytosis (PLCH), after nine years since the first suspicion of PLCH. Furthermore, he was diagnosed with secondary pulmonary hypertension (PH) caused by progressed PLCH by right heart catheterization. At 59 years of age, he was diagnosed with panhypopituitarism, and persistent hormone replacement therapy was subsequently started by an endocrinologist. After the initiation of oxygen therapy and treatment with a combination of sildenafil and warfarin, an estimated pulmonary artery systolic pressure reduced 97.9 to 64.0 mmHg. We believed this is a rare case of PLCH with irreversible central nervous system (CNS) disorder in whom severe PH developed due to a long-term burden of PLCH in a middle-aged male.

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Section: Discussionmentioning

confidence: 89%

Section: Discussionmentioning

confidence: 99%

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Secondary pulmonary hypertension due to pulmonary Langerhans cell histiocytosis accompanied with panhypopituitarism

Miyashita

Yamazaki

Ohta

et al. 2020

Respirology Case Reports

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“…Es más frecuente en la edad pediátrica; en los adultos no se cuenta con evidencia certera respecto a su epidemiología, pero se estima una incidencia anual de 3-4 casos por millón 1,7 .…”

Section: Discussionunclassified

“…La histiocitosis de células de Langerhans (HCL) de hueso, también llamada granuloma eosinófilo, fue descrito por primera vez en 1940. En 1953 se clasificó junto a con la histiocitosis X, la enfermedad de Hand-Schüller-Christian y la enfermedad de Lettere-Siwe [1][2][3] . Es una patología infrecuente, de carácter benigno, con una incidencia anual de 3-4 casos por millón, con predominio en pacientes pediátricos 4 .…”

Section: Antecedentesunclassified

Histiocitosis de Langerhans en el cráneo. Informe de un caso

Higuera-Cárdenas¹,

Román-Matus²,

Martínez-Contreras³

et al. 2018

CIRU

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Potential indication of chemotherapy for hypodipsia and arginine vasopressin deficiency secondary to hypothalamic-pituitary Langerhans cell histiocytosis: a case report and literature review

Ota,

Sato,

Nakano

et al. 2024

Clin Pediatr Endocrinol

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Hypothalamic-pituitary Langerhans cell histiocytosis (HP-LCH) is often associated with arginine vasopressin deficiency (AVD). Patients with AVD caused by HP-LCH rarely develop an impaired osmotic threshold for thirst (OTT). Improvement in OTT among such patients has not been reported in the literature. To our knowledge, here we report the first case of AVD due to HP-LCH in which hypodipsia resolved during chemotherapy. A nine-year-old Japanese girl presented with polydipsia, polyuria, anorexia, and hypernatremia (149.8 mEq/L) and was diagnosed with AVD secondary to HP-LCH. Visual analog scale examination showed a reduced OTT following the water deprivation test. During chemotherapy for Langerhans cell histiocytosis (LCH), serum sodium concentrations became stable between 138.9 and 142.9 mEq/L under the replacement of desmopressin. Repeated visual analog scale examinations showed that she experienced a sense of thirst at a serum sodium concentration of 142.3-144.6 mEq/L, at which she did not experience any thirst prior to the initiation of chemotherapy. These data suggest that chemotherapy directly improved the OTT in our patient. Improved mechanical compression or infiltration of the hypothalamus related to OTT may lead to the recovery of the sense of thirst. This report highlights the potential role of chemotherapy for solitary HP-LCH in patients with hypodipsia and AVD.

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Adult-onset Langerhans cell histiocytosis presenting with adipsic diabetes insipidus, diabetes mellitus and hypopituitarism: A case report and review of literature

Cited by 5 publications

References 17 publications

Secondary pulmonary hypertension due to pulmonary Langerhans cell histiocytosis accompanied with panhypopituitarism

Secondary pulmonary hypertension due to pulmonary Langerhans cell histiocytosis accompanied with panhypopituitarism

Histiocitosis de Langerhans en el cráneo. Informe de un caso

Potential indication of chemotherapy for hypodipsia and arginine vasopressin deficiency secondary to hypothalamic-pituitary Langerhans cell histiocytosis: a case report and literature review

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