A Case of Kikuchi – Fujimoto Disease.

Kikuchi – Fujimoto Disease (KFD) or histiocytic necrotizing lymphadenitis is a rare benign, self-limited cervical lymphadenitis of unknown etiology. Kikuchi first described the disease in 1972 in Japan. Fujimoto and colleagues independently described Kikuchi’s disease in the same year. It pre-dominantly affects young women and can closely mimic infective and immunological disorders. In this article a case of KFD in a young female is described.

Linfadenitis necrotizante histiocitaria: Comunicación de 3 casos / Histiocytic necrotizing lymphadenitis: report of 3 cases

Histiocytic necrotizing lymphadenitis (also known as Kikuchi-Fujimoto's disease) is an uncommon, benign and self-limiting lymph disease. We report three patients aged 14, 22 and 50 years, who presented with fever and cervical lymphadenopathy, accompanied by skin lesions and joint pain in two of the three cases. One of the patient's sister suffered from histiocytic necrotizing lymphadenitis. Laboratory abnormalities varied and findings included leucopenia, relative neutrophilia, elevated C reactive protein, erythrocyte sedimentation rate and ferritin. Basic laboratory screening tests were performed on all patients to rule out autoimmune and infectious diseases. Lymph node biopsy and subsequent pathological examination were essential to establish the diagnosis. All patients received antibiotics at some point of their hospital stay. Two patients required glucocorticoid treatment, while the remaining case experienced a spontaneous recovery. Its pathogenesis is still unknown, but clinical and histopathological studies suggest a connection with autoimmune diseases. There is no established treatment, but apparently the disease responds to the administration of glucocorticoids.

Enfermedad de Kikuchi-Fujimoto: a propósito de un caso / Kikuchi-Fujimoto disease: A propos of a case

La enfermedad de Kikuchi Fujimoto es una forma infrecuente de linfadenitis. En la actual provincia de Mayabeque antes provincia de La Habana no se recoge ningún caso con el referido diagnóstico. Se comunica una paciente femenina de 25 años, color de la piel negra, con diagnóstico de enfermedad de Kikuchi-Fujimoto, que presentaba adenopatías predominantemente en región cervical izquierda. Se realizaron estudios de hematología general, serología viral, exéresis y biopsia del ganglio. La biopsia ganglionar mostró abundantes histiocitos, inmunoblasblos, necrosis celular con polvo nuclear y cariorrexis, así como ausencia de neutrófilos y eosinófilos. Evolucionó favorablemente y actualmente se sigue en consulta de hematología

Kikuchi-Fujimoto disease is a rare form of lymphadenitis. In the current province of Mayabeque formerly Havana Province no cases have been taken up with the referred diagnosis. It is communicated a female patient of 25 years old, black skin color, diagnosed with Kikuchi-Fujimoto disease, who predominantly presented adenopathies in the left cervical region. Studies of general hematology, viral serology, exeresis and ganglion biopsy were performed. ganglionar biopsy showed abundant histiocytes, immunoblasts, cell necrosis with nuclear dust and karyorrhexis, as well as absence of neutrophils and eosinophils. The patient progressed favorably and is currently being monitored in hematology consultation

Enfermedad de Kikuchi-Fujimoto / Kikuchi-Fujimoto’s disease

La Enfermedad de Kikuchi-Fujimoto, también llamada Linfadenitis Histiocítica Necrosante, es un sindrome benigno y autolimitado descubierto por primera vez en 1972 por Kikuchi y por Fujimoto, dos patólogos japoneses. Es una enfermedad principalmente descrita en mujeres jóvenes hasta los 40 años de edad que se ha encontrado en todos los grupos étnicos y que puede presentarse en una proporción menor en hombres. La etiología es desconocida, aunque se ha asociado a ciertos microorganismos, y son las infecciones junto con el linfoma los principales diagnósticos diferenciales de esta enfermedad, caracterizada por linfadenopatía cervical con dolor, fiebre leve y síntomas sistémicos. El estudio histopatológico de los ganglios linfáticos permite diferenciar esta enfermedad de otras más graves, aunque es indistinguible histológicamente del Lupus Eritematoso Sistémico con el cual puede presentarse concomitantemente.

Kikuchi-Fujimoto’s disease, also called Histiocytic Necrotizing Lymphadenitis is a benign, self-limited syndrome. It is a disease mainly described in young women under 40 years of age, it has been found in all ethnic groups and may occur in a lesser extent in men. The etiology is not known, although it has been associated with some microorganisms. The differential diagnoses of this disease are infections and lymphoma. This disease is, characterized by cervical lymphadenopathy with pain, mildfever and systemic symptoms. Histopathological study of lymphnodes differentiate this disease from others more serious, although histologically it’s indistinguishable of Systemic Lupus Erythematosus with which can occur concomitantly.

Kikuchi-Fujimoto disease in fine needle aspiration smears: A clinico-cytologic study of 18 pediatric cases and correlation with 68 adult patients.

Objective: Kikuchi-Fujimoto disease (KFD) is a self-limiting disorder which usually affects young women. There are only a few studies on pediatric KFD patients and their fine-needle aspiration (FNA) cytodiagnosis. We report a series of pediatric KFD patients diagnosed by FNA cytology and compare them with adults. Materials and Methods: By routine FNA cytology and through retrospective review smears initially diagnosed as reactive hyperplasia of lymph node during the years 2004-2009, 18 pediatric and 68 adult KFD cases were detected. The clinico-cytologic features of these two groups were compared. Results: The age of the pediatric patients ranged from 6 to 18 years with a median of 13.5 years and adult cases were aged 19 to 54 years with a median of 30 years. Only 1 pediatric case (5.6%) and 20 (29.4%) adult cases were initially diagnosed as KFD (P = 0.0604). Arab:non-Arab ratios were 4.3:1 among the pediatric patients and 1:1.5 for the adults (P = 0.0043). FNA smears were highly cellular in 4 (22.2%) pediatric cases and 37 (54.4%) adult cases (P = 0.0180). More than 5% Kikuchi histiocytes was present in 8 (44.4%) pediatric and 49 (72.1%) adult cases (P = 0.0474). No significant difference was observed in male: female ratio, frequency of head & neck lymphadenopathy, time (season) of presentation, and presence of neutrophils and capillary networks, etc. Conclusions: Besides observation and interpretation errors, a significant difference between the two groups in respect of some clinico-cytomorphological features could have influenced the routine cytodiagnosis leading to lower pickup rate of pediatric KFD cases as compared to adults.

Kikuchi-Fujimoto disease: an unusual association with acute renal failure

Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis of unknown etiopathogenesis, is a self-limited disease which frequently appears as feverish lymphadenomegaly, thus creating the need for differential diagnosis with lymphoma, systemic lupus erythematosus (SLE), infectious mononucleosis, cat-scratch disease, and toxoplasmosis with lymphonodal impairment. However, there are cases in which it may evolve with complications such as aseptic meningitis, cerebellar ataxia, and aseptic myocarditis. We are presenting a case of a 24-year-old man who had an initial picture of arthralgia, evening fever and adenomegaly. Kikuchi disease was diagnosed through lymph node biopsy with immunohistochemistry and evolves with severe systemic manifestations, such as pericarditis with cardiac tamponade, pneumonitis, hepatitis, and acute kidney failure - the latter has not been reported in literature yet. There was significant improvement of the clinical picture with prednisone.

Childhood kikuchi-fujimoto disease.

To investigate the clinical features of Kikuchi-Fujimoto disease (KFD) and the relationship between viral infection and this disease in children will be better as Kikuchi-Fujimoto disease (KFD) is a lymphadenopathy. The aim of study is to investigate the clinical features of KFD and the relationship. The age, gender, clinical features and aetiopathogenesis of 36 Chinese children with FKD were reviewed, and the viral antigens were detected. Mean age was 10.1 ± 2.8 yr with a male to female ratio of 1.8:1. Fever and lymphadenopathy were the most common complaints, noted in 23 and all cases respectively. Skin rash and hepatosplenomegaly were also noted. Leukopenia, anemia, thrombocytopenia and raised ESR were noted in 21, 6, 4 and 31 cases respectively. Epstein-Barr virus (EBV) IgM and IgG was positive in 1 and 24 of 29 cases respectively. Antigens of EBV and herpes simplex virus 2 (HSV 2) were found in the biopsy tissue section from 2 and 1 case respectively. Autoantibodies were noted in 3 of 15 cases. Steroid hormones were administrated for 19 cases with good efficacy. These results imply that children with lymphadenopathy and/or fever may have KFD and thus excisional biopsy of lymph nodes should be performed earlier on. A hyperimmune reaction of immune cells to EBV and HSV2 may play a role in the pathology of KFD.

Enfermedad de Kikuchi-Fujimoto: presentación de un caso clínico / Kikuchi-Fujimoto disease: case report

La enfermedad de Kikuchi-Fujimoto es una enfermedad de los ganglios linfáticos, habitualmente benigna y autolimitada, que afecta principalmente a adultos jóvenes y se caracteriza poradenopatías (en general cervicales) e hipertermia persistente. Debe diferenciarse de otras causas, infecciosas o tumorales, que cursan con cuadro clínico similar. Su diagnóstico es anatomopatológico (linfadenitis necrotizante histiocitaria) y su tratamiento sintomático. Se presenta un caso clínico de enfermedad de Kikuchi-Fujimotoen una niña de 12 años y se revisa la bibliografía mundial.

Tumor cervical por enfermedad de Kikuchi: Una entidad clínica a la que no estamos habituados. Caso clínico / Histiocytic necrotizing lymphadenitis: Report of three cases

Histiocytic necrotizing lymphadenitis (HNL), also known as Kikuchi 5 disease is a rare condition of unknown etiology. Patients present with cervical lymph node enlargement, fever and malaise. The diagnosis is made by excision biopsy. However, this entity must be distinguished from both reactive processes and malignant tumors such as lymphoma. The clinical course is self limited with spontaneous resolution within a few months. We report three patients with the disease. A 37 year-old woman with a 4 months history of a painless submaxillary mass of 2.5 cm diameter, attached to the deep tissues of the neck. The mass was excised and the biopsy report was HNL. After 26 months of follow up, the patient is asymptomatic. A 30 year-old woman with a history of 2 months of a painless lateral cervical mass and aspiration biopsy was reported as suspicious for lymphoma. An excision biopsy was performed, that was reported as HNL. In both patients, lymphoma was ruled out by immunohistochemistry. A 33 year-old woman with a 3 weeks history of an asymptomatic lateral cervical mass. Biopsy was reported as HNL. This condition must be included in the differential diagnosis of cervical asymptomatic masses. The clinician must be aware of it to avoid long-term, costly treatments.

Clinicopathological study of Kikuchi's disease.

Kikuchi's disease (KD) is a necrotizing lymphadenopathy with a self-limiting course commonly affecting young people. We conducted a clinico-pathological study of 20 cases of KD received at our department between July 2000 and December 2003. Our study confirmed that KD commonly involves the cervical lymph nodes of young adults, with a female predominance. One patient had associated cutaneous involvement. The hematological parameters were non-specific. The histological features were distinctive and included easy fragmentation on handling, karyorrhexis, crescentic histiocytes and the absence of neutrophils. The differential diagnosis on histology is also discussed.

Linfadenitis necrotizante histiocitaria o enfermedad de Kikuchi-Fujimoto: a propósito de 2 observaciones / Histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoti disease: apropos of two observations

La linfadenitis necrotizante histiocitaria o enfermedad de Kikuchi-Fujimoto es un proceso benigno poco frecuente. Se presentaron 2 casos diagnosticados con poco tiempo de aparición entre uno y otro. El primero fue una mujer blanca, de 26 años, con astenia, pérdida de peso, sudación nocturna, escalofríos, prurito generalizado y anorexia. Al realizar examen clínico se demostró la presencia de adenopatías y en los exámenes de laboratorio no se halló nada significativo. Se obsevaron varias estructuras ganglionares pequeñas con preservación parcial de la citoarquitectura y cambios hiperplásticos ligeros. El segundo paciente, un hombre negro, de 34 años, que se presentó con fiebre vespertina, escalofríos, odinofagia, cefalea y dolores articulares. Se observó orofaringe enrojecida con aumento del tamaño de las amígdalas, sin exudados y adenopatías en la cadena lateral derecha del cuello y submaxilares. No se encontró ningún resultado anormal en los estudios de laboratorio y en el examen histológico del ganglio se observó preservación casi total de su arquitectura, con cambios hiperplásticos corticales y paracorticales. Se observó remisión clínica espontánea en ambos enfermos.

Pyrexia of unknown origin with neutropenia, cervical lymphadenopathy, pneumonia and marrow hypoplasia.

A 56 years adult male presented with fever for 3 weeks with neutropenia and cervical lymphadenopathy with left sided pneumonitis. Histopathology of lymphnode was consistent with Kikuchi's Necrotizing Lymphadenitis. Kikuchi's disease is usually a self- limiting illness characterized by pyrexia, neutropenia and cervical lymphadenopathy in young women of Asian decent. This often leads to the misdiagnosis of lymphoma or tuberculosis. The notable feature here is an older male presented with severe neutropenia and pneumonia with hypoplastic marrow.

Necrotizing non-granulomatous lymphadenitis: a clinicopathologic study of 40 Thai patients.

The purpose of this study was to describe the clinicopathological features of 40 cases of necrotizing non-granulomatous lymphadenitis in Thai patients. The clinical features, histomorphology and special stains were evaluated in 40 Thai patients from the pathology records of King Chulalongkorn Memorial Hospital from January 2001 to December 2003 in those diagnosed as having necrotizing non-granulomatous lymphadenitis. Of the 40 patients, 17 cases (42.5%) had Kikuchi-Fujimoto disease (KFD), 8 cases (20%) had tuberculosis (TB) lymphadenitis and 1 case (2.5%) had systemic lupus erythematosus (SLE) with associated lymphadenitis. Fourteen cases (35%) did not have a specific diagnosis due to a lack of follow-up data. KFD most commonly occurs in young women, and is characterized by the presence of coagulative necrosis and karyorrhexis often centered in the paracortex, an absence of neutrophils and plasma cells, proliferation of various cells composed of lymphocytes, histiocytes, immunoblasts and plasmacytoid monocytes and the absence of a granuloma. Tuberculous lymphadenitis usually occurs in women with a mean age of 34.25 years. The lymph nodes reveal extensive coagulative necrosis involving the cortex, paracortex and medulla, proliferation of mixed inflammatory cells, including neutrophils, lymphocytes and plasma cells in the necrotic area and the presence of proliferating histiocytes at the periphery of the necrotic area. The lymph nodes of SLE-associated lymphadenitis reveal large numbers of plasma cells and hematoxylin bodies. We suggest that necrotizing non-granulomatous lymphadenitis is not specific for any disease, but rather a common histologic change found in diseases, such as TB, SLE, and KFD. Further investigation to obtain a definite diagnosis should be done for appropriate treatment.

Linfadenite necrotizante nistiocítica / Histiocytic necrotizing lymphadenitis

A doença de Kikuchi-Fujimoto é uma desordem de condição clínico-patológica benigna e autolimitada, que afeta predominantemente mulheres jovens. Acomete preferencialmente a cadeia linfonodal cervical e comumente está associada a sintomas inespecíficos, como febre e artralgia. Este trabalho tem por objetivo relatar e evidenciar a associação entre as patologias lúpus eritematoso sistêmico e doença de Kikuchi-Fujimoto, através do caso de paciente do sexo feminino, branca, 16 anos de idade, com quadro de linfadenopatia cervical, febre e artralgia e cujo anatomopatológico de biópsia linfonodo cervical evidenciou linfadenite necresante histiocítica. As provas imunológicas foram positivas para lúpus eritematoso sistêmicos e houve resposta ao tratamento com corticosteróides.

Kikuchi-Fujimoto disease--a case report.

The etiology of cervical lymphadenopathy is multifactorial including infections and tumoral lesions. Histiocytic necrotising lymphadenitis or Kikuchi-Fujimoto's disease is a rare cause of cervical lymphadenopathy which does not have specific clinical signs. We report herewith a case of Kikuchi's disease (KD) diagnosed by histopathology.

Kikuchi's disease.

An 11-year-old girl presented with high grade, intermittent fever and cervical lymphadenopathy. She had multiple enlarged left cervical lymph nodes. The examination of other systems was normal. Workup for sepsis, malignancy and autoimmune disease were negative. VA-IgM for EBV was positive and histopathology of the lymph node was consistent with Kikuchi's disease.

Enfermedad de Kikuchi - Fujimoto: reporte de un caso / Kikuchi - Fuijimoto disease: report a case

La enfermedad de Kikuchi o Kikuchi Fujimoto o linfadenitis histiocítica necrotizante, es una rara y habitualmente benigna enfermedad que se presenta con fiebre y adenopatías cervicales usualmente en mujeres jóvenes. La etiología es desconocida, aunque hay evidencias de su origen viral y la semejanza del grupo poblacional afectado con el lupus eritematoso sistémico ha alentado, en base a estudios celulares, la sospecha de que el síndrome de Kikuchi refleja una condición Lupus - símil auto limitada y causada por linfocitos transformados por una infección viral. Inicialmente descripta en Japón, la enfermedad de Kikuchi Fujimoto se ha reportado en diferentes grupos étnicos y raciales, aunque ha sido predominantemente descripto en Asia. La enfermedad puede ser sospechada por la clínica pero se necesita de la anatomía patológica para su confirmación.