Congenital Letterer-Siwe disease (reticuloendotheliosis) in a term stillborn infant

Ahnquist, Gerhard; Holyoke, John B.

doi:10.1016/s0022-3476(60)80142-4

Cited by 34 publications

(5 citation statements)

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“…However, in contrast to our case, there was extensive subperiosteal bone involvement but no gross or histological changes in the brain. Other congenital cases were described by Ahnquist et al (1960) 3 and Hamilton in 1961. 2 The autolysis, in our case, was very severe precluding detailed histologic examination of the body tissues.…”

Section: Discussionmentioning

confidence: 96%

“…Langerhans cell histiocytosis (LCH) manifests with a heterogeneous spectrum of clinical presentations but is biologically and histopathologically uniform, resulting from the clonal proliferation of Langerhans cells. The disease has gone by several names including Hand–Schüller–Christian disease, Abt-Letterer–Siwe disease, eosinophilic granuloma, histiocytosis X 1-3 which are obsolete since 1987 after the Histiocyte Society grouped all of these under the term “Langerhans cell histiocytosis.” 4 Clinical presentations vary from solitary bone lesions to more severe multifocal or disseminated forms. Four different groups of LCH are defined depending on the number of organs (or systems) involved, single system (SS), lung involvement (LCH lung+), and the involvement of 1 of the 3 following risk organs (ROs): liver, spleen, and bone marrow (LCH MS-RO+, LCH MS-RO−).…”

Section: Introductionmentioning

confidence: 99%

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Unique Case of Congenital Langerhans Cell Histiocytosis Presenting as Intrauterine Fetal Demise

et al. 2022

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Congenital Langerhans cell histiocytosis (LCH) (formerly called Letterer–Siwe disease) is characterized by a clonal proliferation of Langerhans cells occurring in children at birth and manifests typically with multifocal cutaneous lesions, hepatosplenomegaly, lymphadenopathy, pulmonary lesions, and destructive osteolytic bone lesions. We present a case of LCH involving multiple systems high-risk organs (LCH MS-RO+), in a 32-week stillborn from a 20-year-old G2A1. The fetus was mildly hydropic and pale. Apart from maceration, the skin showed multiple targetoid lesions over the face, trunk, and limbs. There was hepatosplenomegaly and a pale brain. The placenta was large and bulky. Despite severe autolysis, histological examination showed disseminated histiocytes with multinucleated giant cells in the skin, lungs, thymus, mesenteric lymph nodes, spleen, and brain. By immunohistochemistry, the histiocytes were positive for S100, CD1a, and Langerin (CD207), confirming the diagnosis of LCH. There was extramedullary hematopoiesis in the spleen, brain, and placenta. Targeted next-generation sequencing performed on thymic DNA did not show the BRAF p.V600E variant but did show the MAP2K1 p.F53_Q58delinsL. Infants with LCH pose a diagnostic challenge due to their heterogeneous presentations. Our case is unusual in that the newborn presented with severe multiorgan involvement including brain and intrauterine death. LCH is still poorly understood requiring further genetic and molecular studies.

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Section: Discussionmentioning

confidence: 96%

Section: Introductionmentioning

confidence: 99%

Unique Case of Congenital Langerhans Cell Histiocytosis Presenting as Intrauterine Fetal Demise

et al. 2022

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“…Some studies have reported an infectious origin in the etiology of Histiocytosis X: Dargeon [26] isolated bacteria in two patients and Ahnquist and Holyoke [2] described congenital Histiocytosis X after poliomyelitis and influenza vaccination during pregnancy. The spontaneous resolution in some cases, the response in some cases to antibiotics and antituberculous drugs [7,16,22,26] and to irradiation at low doses were used as arguments in favour of an inflammatory origin.…”

Section: Chemotherapymentioning

confidence: 99%

“…Males appear to be more affected than females: in extensive studies[33,68,76,88,100] the ratio 6:9 = 2: I is given. Some authors report congenital cases[2,26,68,76,78, 95]. Consequently, Histiocytosis X mainly occurs in the first years of life.…”

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confidence: 99%

Histiocytosis X

Bökkerink

Vaan

1980

Eur J Pediatr

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“…Los hallazgos clinicos encontrados fueron muy variados, hecho resaltado por numerosos autores (9,10,11,12,13,14,15,16,17). Asi, los mas frecuentes (tabla N?…”

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Salmonellosis por Salmonella Newington: seis casos en lactantes

R¹,

G²

1973

Rev. chil. pediatr.

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La Histiocitosis X es una enfermedad de etiologia desconocida (1), cuyo nombre fue dado por Lichtenstein (2), quien demostr6 que el Granuloma eosinofilo, la enfermedad de Hand Shuller Christian (3) y el Letterer Siwe, eran distintas expresiones de una misma alteraci6n histo!6gica, caracterizada por proliferati6n de histiocitos con la presencia, en mayor o menor grado de eosinofilos (4, 5). El mero hallazgo histo!6gico no es suficiente para diagnosticar esta patologia, ya que la proliferation histiocitaria no es en absolute especifica, pudiendo encontrarse en numerosos otros cuadros (6), resaltando asi, una vez mas, la importancia que tiene la estrecha colaboracion del clinico y el pat61ogo para poder realizar un buen diagnostico, tratamiento adecuado y formular un pron6stico.Esta publicaci6n presenta una revisi6n de 23 pacientes tratados en el hospital Luis Calvo Mackenna, entre los anos 1952-1972 y que se encontraban bajo los diagnosticos de: Granuloma eosin6filo, Hand Schuller Christian, Letterer Siwe e Histiocitosis X, mostrando su evolution, pronostico y resultados de los tratamientos efectuados.MATERIAL.-A los 23 pacientes se les realizo anamnesis, examen fisico completo y estudios de laboratorio: hemograma, VHS, orina completo, PPD U, mielograma, radiografias de esqueleto y torax y en algunos, biopsia. Los pacientes, asi estudiados, se distribuyeron segun la clasificion de Oberman (7) modificada por Ekert y Campbell (8), que consta de cuatro grupos, a saber: A) Lesi6n osea unica (B) Lesion osea multiple, C) Lesion osea y compromiso de tejidos blandos y D) Compromiso exclusive de tejidos blandos. RESULTADO Y COMENTARICW. Entre los cldsicos grupos descritos existen multiples cuadros intermedios, de alii el valor que le asignamos a la clasificacion de Oberman que los agrupa de acuerdo al numero de lesiones y difusion del proceso.Analizando los datos obtenidos se aprecia que 14 pacientes eran mujeres y 9 hombres, hecho no coincidente con estadisticas extranjeras en las que predominan los varones (9). A su vez, en el sexo femenino la letalidad es mayor, 29%, comparada con un 11% en varones, situation destacada por Avery y otros investigadores (9).La edad de aparicion del cuadro fue muy variable, desde el nacimiento hasta los 11 anos, agrupandose la gran mayoria en los tres primeros anos de vida, destacando el primero y entre el segundo y tercer ano de vida (figura W 1).

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Congenital Letterer-Siwe disease (reticuloendotheliosis) in a term stillborn infant

Cited by 34 publications

References 13 publications

Unique Case of Congenital Langerhans Cell Histiocytosis Presenting as Intrauterine Fetal Demise

Unique Case of Congenital Langerhans Cell Histiocytosis Presenting as Intrauterine Fetal Demise

Histiocytosis X

Salmonellosis por Salmonella Newington: seis casos en lactantes

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