Two Cases with Clinical Diagnosis of Subacute Necrotizing Lymphadenitis Successfully Treated with Shosaikoto / 日本東洋医学雑誌

We experienced 2 patients with clinical diagnosis of subacute necrotizing lymphadenitis who were successfully treated with shosaikoto. Case 1 is a 37-year-old woman. Left neck swelling and pain appeared from 12 days before, and temperature went up thereafter. Antibiotics were administered at another hospital, but she did not improve. There were multiple swollen lymph nodes of the left neck, and blood test showed decreased white blood cells and increased LDH. So we clinically diagnosed her as subacute necrotizing lymphadenitis. After administration of shosaikoto, fever resolved, and cervical swelling also markedly improved on the third day. On day 10, both symptom and findings disappeared. Case 2 is a 12-year-old man. Left neck swelling and pain appeared and temperature went up from 8 days before. An antibiotic was administered at another hospital, but he did not improve. There were multiple swollen lymph nodes of the left neck, and blood test showed decreased white blood cells and increased LDH. So we clinically diagnosed him as subacute necrotizing lymphadenitis, and administered shosaikoto. Then fever resolved on the third day, and cervical swelling diminished and tenderness disappeared on day 10. On day 18, both symptom and findings disappeared.

Discrimination Between Childhood Subacute Necrotizing Lymphadenitis and Nonspecific Cervical Lymph Hyperplasia for Earlier Diagnosis / 소아알레르기및호흡기학회지

PURPOSE: We conducted this research to make an earlier diagnosis and identify better treatment for Kikuchi-Fujimoto disease (KFD) by comparing clinical findings with nonspecifically enlarged cervical lymph nodes in children. METHODS: Nineteen patients were diagnosed with KFD by tissue pathology from a fine needle aspiration biopsy and/or excisional biopsy and were compared with the clinical, radiological, and pathological findings of reactive hyperplasia. RESULTS: The average onset age of onset for patients with KFD was 11.8+/-3.61 years, and the male to female ratio was 1:1.1, whereas patients with reactive hyperplasia were 11.8+/-5.96 years, and the male to female ratio was 1.7:1. Patients with KFD suffered more from fever than patients with reactive hyperplasia (68% vs. 13%, P=0.002). Patients with KFD showed perinodal infiltration (P=0.001) and necrosis on computed tomography, whereas patients with reactive hyperplasia did not show any of these findings. Ultrasonographic findings were similar between the two study groups. In contrast, the histopathological examinations of biopsied cervical lymph nodes were enormously helpful for distinguishing the findings of KFD from those of patients with reactive hyperplasia. CONCLUSION: We recommend a histopathological examination to distinguish KFD from reactive hyperplasia in children with significantly enlarged cervical lymph nodes.

Clinical analysis with subacute necrotizing lymphadenitis / 国际儿科学杂志

Objective To investigate etiological factors, clinical characteristic and related laboratory findings of subacute necrotizing lymphadenitis and improve the acknowledgement of subacute necrotizing lymphadenitis. Methods We analyzed 14 cases of subacute necrotizing lymphadenitis who had been diagnosed by lymph node biopsy retrospectively. Results The most of patients were school children with mean age of 10.4 ± 2.9) years. The ratio of male to female was 1.8∶1 .The main clinical symptoms were that lymph nodes were swelling and pressing-pain in 14 cases (100%) and fever in 13 cases (92.9%), rash in 2 cases (14.3%),hepatosplenomegaly in 2 cases (14.3%).The white blood cell count was (2.3 ～ 7.4) × 109/L in 14 cases and average count was (3.90 ± 0.35) × 109/L. The white blood cell count showed leukopenia in 10 cases (71.4%) and none showed heighten. Twelve cases were tested by CRP and the result of 8 cases elevated slightly (10 ～ 30 mg/dl). Thirteen cases underwent ESR examination and the result of 11 cases showed higher than that of normal level (20mm/h)(82.6%). The average ESR risen value was 40mm/h and topmost value was 70 mm/h. Eight cases underwent anti-nuclear antibody test and 2 cases of them were positive. Twelve cases underwent EB virus antibody test and 4 cases of them were positive. Eleven cases underwent blood Mycoplasma-IgM examination and one of them was positive. Six cases underwent coxsackie virus antibody and one of them was positive.Coxsackie and EB virus infected in one case. Lymph node biopsy was found necrotic fragments in different sizes. The cellular hyperplasia was found around the necrotic area and plasma cells, neutrophils, and eosinophils were found rarely in these areas. The DNA of EB virus was negative in lymph nodes. Eight cases of all were treated by prednisone and effect showed well 1.5 ～ 2.0 mg/(kg·d)). One of all was recurrence and the rest recovered well. Blood routine examination was used when they were under a stable condition, five cases of them showed normal white blood cells. Conclusion Those with fever of unknown origin combined with lymphadenectasis are suggested to have an early lymph node biopsy. The process is self-limited and glucocorticoid therapy is effective to those impatients. The long-term follow up is required as recurrence and development of SLE have reported. EB virus was not an etiological factor of SNL. Leucocyte can be used as one of the indicators of improvement.

A Case of Systemic Lupus Erythematosus Involving the Kidneys in a Patient with IgA Nephropathy / 대한류마티스학회지

Renal involvement in systemic lupus erythematosus (SLE) is a typical manifestation of the disease. The occurrence of non-lupus nephritis, especially IgA nephropathy (IgAN), in patients with SLE has rarely been reported. We describe the case of a 21-year-old woman who was diagnosed with IgAN and subacute necrotizing lymphadenitis, and her renal lesion biopsy was typical of lupus nephritis (ISN/RPS Class III). Although IgAN and lupus nephritis share some common physiopathological characteristics, their laboratory, histopathologic findings, and the extra-renal clinical manifestations are different and support a different pathogenesis. Our case highlights the importance of a renal biopsy in patients with lupus and urinary alterations despite underlying IgAN. A correct diagnosis would permit the most appropriate immunosuppressive treatments to be considered.

Analysis of disease mechanism of subacute necrotizing lymphadenitis in children / 소아과

PURPOSE: The cause of subacute necrotizing lymphadenitis, a rare disease in children, has not been completely clarified. This study was aimed to investigate the disease mechanism by examining clinical, radiologic, and immunohistochemical findings in children diagnosed with subacute necrotizing lymphadenitis after an excisional biopsy. METHODS: We examined 19 lymph node tissue specimens from 17 children diagnosed with subacute necrotizing lymphadenitis at Gyeongsang National University Hospital from March, 1998 to July, 2006. A retrospective survey of the medical records was performed. CT findings were analyzed. Immunohistochemical staining was done on tissues obtained by excisional biopsy from all patients. RESULTS: The patient's age ranged from 5 to 19 years (average age :11.8 years). The main symptoms included a neck mass (17/19), pain in the mass (6/17), and fever (12/19). The palpable lymph nodes were mostly cervical in location; the maximum diameter, which was measured radiologically, was less than 3 cm in all 10 cases. The masses were pathologically divided into proliferative, necrotic, and xanthomatous types. With immunohistochemical staining the masses were divided into lesion (L), perilesion (PL), and necrosis (N). The CD8 staining was stronger than the CD4 staining for all regions in three types. The CD4 staining intensity was mainly increased in the perilesion, and CD8 was mainly increased in the lesion. CONCLUSION: We compared the radiologic findings, clinical symptoms, and pathology to help understand the cause of disease in patients with subacute necrotizing lymphadenitis.

Clinical Characteristics and Atypical Cases of Subacute Necrotizing Lymphadenitis / 감염

BACKGROUND: Subacute necrotizing lymphadenitis (SNL) or Kikuchi-Fujimoto disease, first described in 1972, is a benign process of lymphatic system that consists of lymphadenopathy which may be accopanied by fever, rash and some hematologic alterations. It affects usually young people, mostly women. Even though this disease is self-limited, many cases are misidentified as malignant lymphoma. The purpose of this study is to report the clinical characteristics, many laboratory tests and pathologic finding and to elaborate the criteria that are useful in distinguishing from fever cause. We are going to present some rare cases and fatal cases. METHODS: Authors evaluated 116 patients, who were diagnosed as Subacute necrotizing lymphadenitis on excisional biopsy with retrospective chart review at Yonsei Medical Center from January 1993 to May 2000. RESULTS: The mean age was 26.4+/-0.9 years (range : 5~63 years) and the male to female ratio was 1 : 2.28. The most common symptoms were cervical lymphadenopathy (89.7%) and fever (57.8%), and the duration of symptom was generally less than 8 weeks. Leukopenia (74.4%), relative lymphocytosis (33.3%) and DIC (7 cases) were hematologic abnormalities. Recently, we experienced patients showing atypical clinical findings which were pancytopenia, DIC (disseminated intravascular coagulopathy), hypotension or debilitating symptoms. Steroid pulse or high dose steroid and immunosuppressant therapy were performed in atypical cases. Sj gren some cases were rarely associated with systemic diseases such as systemic lupus erythematosus (4 cases), Sj gren disease (2 cases), rheumatoid arthritis (3 cases), aseptic meningitis (2 cases) or adult Still's disease (1 case). Thirteen cases (10.9%) were recurrent, and two cases died. CONCLUSION: Subacute necrotizing lymphadenitis should be considered in the differential diagnosis of fever cause in the patients which had localized cervical adenopathy, unresponsive to antibiotic therapy, especially in young women. We report atypical or fatal cases and need clinical attention to this kind of possibility.

Clinical Features of Subacute Necrotizing Lymphadenitis in Children

PURPOSE: There is a predilection for subacute necrotizing lymphadenitis(SNL) in young Asian women. Few pediatric cases have been reported. This study was performed to evaluate clinical features of children with SNL. METHODS: We retrospectively analyzed clinical features of 23 cases of SNL under 15 years of age identified in Nowon Eulji Hospital from February 1995 to January 2002. Two cases were diagnosed by excisional biopsy and 21 cases by fine needle aspiration. RESULTS: The ages ranged from 14 months to 14 years(mean 8.1+/-3.8 years). The male to female ratio was 1 : 1.6. Ten cases(10/23) were diagnosed in 2000. The onset was more frequent in spring. The common presenting symptoms were neck mass(22/23), pain(12/20) and fever(8/18). Fever subsided within two weeks in seven out of eight and lymphadenopathy resolved within five months in 14 out of 15. The mean WBC count was 7,664+/-3,454/mm3. Elevated levels of ESR(10/ 12) and lactate dehydrogenase(5/6) and positive reaction for CRP(3/4) were frequent. Radiologically measured greatest diameter of the enlarged lymph nodes was less than 2 cm in 14 out of 15. One case occurred in a child with alopecia areata and one case with type 1 diabetes. Clinical outcomes were good in 23 cases. CONCLUSION: SNL should be considered in children with cervical lymphadenopathy regardless of fever or pain. SNL seems not rare in pediatric groups in Korea.

A Case of Subacute Necrotizing Lymphadenitis with Hepatic Complication in an 11-year-old Boy / 대한소아소화기영양학회지

Subacute necrotizing lymphadenitis was first reported by Kikuchi and Fujimoto in 1972. Young females no more than 30 years of age are mainly affected. It usually manifests as fever and cervical lymphadenopathy. We experienced one case of subacute necrotizing lymphadenitis with hepatic complication in an 11-year-old boy. Symptoms presented were URI signs, diarrhea, headache, and weight loss along with fever and cervical lymphadenopathy. Elevated serum AST/ALT levels were also noted up to 682/1560 (IU/L) and were normalized within one month. We performed aspiration biopsy of the liver twice (at admission and 5 months thereafter). The hepatic histopathologic findings were nonspecific.

Three Cases of Subacute Necrotizing Lymphadenitis in Childhood

Subacute necrotizing lymphadenitis was first reported by Kikuchi and Fujimoto in 1972. Young females no more than 30 years of age are mainly affected. It usually manifests as fever and cervical lymphadenopathy. We experienced three cases of subacute necrotizing lymphadenitis in an 11-year-old boy, a 12- year-old boy and an 11-year-old girl. Symptoms presented were URI signs, diarrhea, headache and weight loss along with fever and cervical lymphadenopathy. Fever persisted from 9 to 30 days and did not respond to antibiotics. We performed aspiration biopsy of cervical lymph node which was consistent with subacute necrotizing lymphadenitis in all cases. We used oral steroid in one case. Fever and lymphadenopathy subsided spontaneously within one month. There were no other complications.

Three Cases of Subacute Necrotizing Lymphadenitis in Childhood

Subacute necrotizing lymphadenitis was first reported by Kikuchi and Fujimoto in 1972. Young females no more than 30 years of age are mainly affected. It usually manifests as fever and cervical lymphadenopathy. We experienced three cases of subacute necrotizing lymphadenitis in an 11-year-old boy, a 12- year-old boy and an 11-year-old girl. Symptoms presented were URI signs, diarrhea, headache and weight loss along with fever and cervical lymphadenopathy. Fever persisted from 9 to 30 days and did not respond to antibiotics. We performed aspiration biopsy of cervical lymph node which was consistent with subacute necrotizing lymphadenitis in all cases. We used oral steroid in one case. Fever and lymphadenopathy subsided spontaneously within one month. There were no other complications.

A Recurred Case of Subacute Necrotizing Lymphadenitis(Kikuchi's Disease) During Childhood

Subacute necrotizing lymphadenitis, or Kikuchi's disease, primarily affects the cervical lyrnph nodes of lung adults and has a self-limited clinical course. Differential diagnoses are malignant lymphoma and systemic lupus erythematosus. Recurrence rate is low as 3.3%, and there have been a few reports describing the recurrence of the disease. The symptoms in almost all recurrent cases were similar to those of the early stages of the disease. The etiology of the disease remains unknown, but various viral infections and autoimmune processes have been postulated to be the cause. A 6-year-old boy presented with a fever of approximately 40C degrees, painless lumps on both sides of the neck, and intermittent abdominal pain for 2 weeks. Subacute necrotizing lymphadenitis was diagnosed 3 years before admission, which was reported by us in 1996. Diagnosis of recurred subacute necrotizing lymphadenitis was confirmed by histological examination of the lymph node. We report a recurred case of subacute necrotizing lymphadenitis during childhood.

The Lymphocyte Subset Change in a Case of Subacute Necrotizing Lymphadenitis

The subacute necrotizing lymphadenitis, also known as Kikuchi-Fujimoto disease, is a benign, self-limited disorder of unknown cause. This rare disease is characterized by fever, lymphadenopathy, leukopenia, and elevated sedimentation rate. This disease preferentially affects young women. The diagnosis of this disease is confirmed by involved lymphnodes biopsy. Evidence for autoimmune dysfunction has been reported, but is usually lacking. According to the review of the literatures, a total of 192 cases of subacute necrotizing lymphadenitis were reported in Korea since 1983 mostly in literatures of pathology or internal medicine and only 7 cases in pediatrics. There was female predominance in a 141:51 ratio. However, among 25 cases of under 20 years of age, there was male predominance in a 16:9 ratio. The patients of under and over 20 years of age were 29 and 82, respectively among the age-known 111 cases of 192 reported. We experienced a 13-year-old boy, who had right cervical lymphadenitis with persisting high temperature and pain on lymph nodes for more than 10 days. An excisional cervical lymph node biopsy was performed and pathologic diagnosis was compatible with subacute necrotizing lymphadenitis. Most antibiotics were not effective, but fever subsided with one day of oral prednisolone therapy. Various laboratory studies for prolonged fever were negative except leukopenia and increased sedimentation rates. The CD4/CD8 ratios in acute and recovery stages were 1.1 and 1.5 respectively, showing mild decrease of CD4/CD8 ratio in the acute stage. Decreased T cell proportions in the acute stage were also noted.

The Lymphocyte Subset Change in a Case of Subacute Necrotizing Lymphadenitis

The subacute necrotizing lymphadenitis, also known as Kikuchi-Fujimoto disease, is a benign, self-limited disorder of unknown cause. This rare disease is characterized by fever, lymphadenopathy, leukopenia, and elevated sedimentation rate. This disease preferentially affects young women. The diagnosis of this disease is confirmed by involved lymphnodes biopsy. Evidence for autoimmune dysfunction has been reported, but is usually lacking. According to the review of the literatures, a total of 192 cases of subacute necrotizing lymphadenitis were reported in Korea since 1983 mostly in literatures of pathology or internal medicine and only 7 cases in pediatrics. There was female predominance in a 141:51 ratio. However, among 25 cases of under 20 years of age, there was male predominance in a 16:9 ratio. The patients of under and over 20 years of age were 29 and 82, respectively among the age-known 111 cases of 192 reported. We experienced a 13-year-old boy, who had right cervical lymphadenitis with persisting high temperature and pain on lymph nodes for more than 10 days. An excisional cervical lymph node biopsy was performed and pathologic diagnosis was compatible with subacute necrotizing lymphadenitis. Most antibiotics were not effective, but fever subsided with one day of oral prednisolone therapy. Various laboratory studies for prolonged fever were negative except leukopenia and increased sedimentation rates. The CD4/CD8 ratios in acute and recovery stages were 1.1 and 1.5 respectively, showing mild decrease of CD4/CD8 ratio in the acute stage. Decreased T cell proportions in the acute stage were also noted.

A Case of Recurrent Subacute Necrotizing Lymphadenitis with Pancytopenia / 대한혈액학회지

We report a case of recurrent subacute necrotizing lymphadenitis with pancytopenia in 21-years-old-woman. She was admitted to our hospital 4-years interval with fever and abdominal pain. Laboratory findings of the last admission showed pancytopenia, such as WBC 700/microliter, hemoglobin 6.0mmol/L (9.7g/dL), hematocrit 28.8%, and platelet 54,000/microliter. Abdominal CT showed hepatosplenomegaly, enlarged conglomerated lymph nodes in splenic hilum, lesser sac, celiac root, and paraaortic areas. Bone marrow biopsy showed hypocellular marrow (20%) with increased number of megakaryocyte, myeloid hyperplasia, and hemophagocytic histiocytes suggesting infectious process. We performed exploratory laparotomy, and pathologic finding revealed subacute necrotizing lymphadenitis-Kikuchi disease-. She was recovered on 26th hospital day with conservative treatment.

Subacute Necrotizing Lymphadenitis in a 3 Year-Old Male Child

Subacute necrotizing lymphadenitis is a benign form of lymphadenitis that was first described in Japan by Kikuchi in 1972. It mainly affects young women and usually manifests as fever and lymphadenopathy. Although it is a benign self-limited lymphadenitis, it has been misdiagnosed as malignant lymphoma. Histologically, involved lymph nodes contain a necrotizing process characterized by patch, well-circumscribed area with eosinophilic fibrinoid material. There is a striking degree of karyorrhexis and an absence of granulocyte with paucity of plasma cell. We expereinced a case of subacute necrotizing lymphadenitis in a 3 year old boy. There was spontaneous resolution of fever and lymphadenopathy, and now he keeps doing well.

Subacute necrotizing lymphadenitis: a collective clinicopathological and immunohistochemical study

Subacute necrotizing lymphadenitis (SNL) is a well documented and unique clinicopathologic entity, although its etiology and pathogenesis have not been clearly established. Microscopically, cortical and paracortical necrotizing lesions with karyorrhexis, abundant nuclear debris and infiltration of large mononuclear cells are characteristic. This study analyzed the common clinical and pathological features of 118 patients with SNL and the nature of the mononuclear cells. Patients were generally young women and revealed cervical lymphadenopathy with tenderness, fever, leukopenia and elevation of the erythrocyte sedimentation rate. Features of the adjacent uninvolved area in the lymph node included a starry sky pattern, follicle centers, sinus histiocytosis or aggregation of foamy histicoytes. There was an inverse relationship between the extent of necrosis and of histocytic infiltration but not between the extent of necrosis and the duration from the onset of symptoms to the diagnosis. Immunohistochemically the infiltrated mononuclear cells of the affected foci were T lymphocytes and histiocytes. The clinical, histological and immunohistochemical features suggest that SNL represents a hypersensitivity reaction to certain infectious agent without forming granuloma.

Necrotizing lymphadenitis--a clinico-pathologic study of 36 cases with immunohistochemical analysis

Thirty-six cases of necrotizing lymphadenitis--including 33 cases of unknown etiology, 1 typhoid lymphadenopathy, and 2 cases of suspicious lupus lymphadenopathy--were clinico-pathologically reviewed and analyzed with immunostaining for s-100 and lysozyme. All cases histologically showed architectural effacement by paracortical lesions composed of nuclear karyorrhexis and mononuclear cell proliferation. Immunohistochemical study revealed proliferation of lysozyme-positive macrophages in the necrotizing areas and an increase in the number of s-100-positive cells in the uninvolved paracortical areas. This observation suggests that necrotizing lymphadenitis may be a common morphologic expression of a T cell-mediated hyperimmune condition induced by diverse etiologies.