Rare Cause Of Lymphadenopathy Research Articles

Kikuchi Fujimoto's Disease: A Strange Cause of Cervical Lymphadenopathy, As A Differential Diagnosis of Nodal Tuberculosis: A Case Report in A Public Hospital - Peru

Kikuchi-Fujimoto disease is a rare cause of lymphadenopathy of unknown etiology to date. Its clinical manifestations are the presence of lymphadenopathies associated with fever, diaphoresis, and night sweats. The diagnosis is made by histopathology and the exclusion of other pathologies, and its management is symptomatic. A case is reported of a 26-year-old woman from Venezuela, from the district of San Juan de Miraflores, department of Lima - Peru, who presented with an increase in the volume of the cervical lymph node in 3 months, very painful associated with night sweats. The diagnosis was established by the anamnesis, clinical picture, laboratory tests, and pathological anatomy; she received symptomatic treatment with a favorable evolution. The clinical, laboratory, and therapeutic aspects of the disease are discussed.

Castleman's Disease: A case report of rare cause of lymphadenopathy in the Head and Neck and review of the literature

Introduction: Castleman's disease is a rare, typically benign, non-neoplastic lymphoproliferative disorder first described by Dr Castleman in the 1954. It can be classified according the number of areas affected as localized (unicentric) or systemic (multicentre) It can also be defined by the microscopic features of the lymph nodes into four categories: hyaline-vascular, plasma cell, mixed and plasmablastic. The most common presentation is the multicentric hyaline vascular type while the thorax and abdomen are the most affected sites. A clinical diagnosis may be suspected but definite diagnosis is achieved with histopathology due to lack of distinguishing radiological features. Management can include surgical resection, chemotherapy and radiotherapy.

Kikuchi–Fujimoto disease: A rare cause of lymphadenopathy and fever

Introduction: Kikuchi–Fujimoto disease (KFD) was originally described in a young woman and is a rare, benign and self-limited condition of unknown cause, usually characterized by cervical lymphadenopathy and fever. The diagnosis of KFD is made by lymph node biopsy and treatment is only symptomatic, with signs and symptoms usually resolving within one to six months. Case Report: A 31-year-old female, rabbit breeder, presented with complaints of fever, facial and hand skin lesions for 10 days. Physical examination revealed nose, malar, scalp, fingers and pinna erythematous papules; also painful bilateral cervical and axillary lymphadenopathy. Laboratory studies showed normocytic/normochromic anemia (hemoglobin 10.4 g/dL), leukopenia (white blood cell count 2.2x103 u/L), neutropenia 44.4% and elevated erythrocyte sedimentation rate 31 mm/h. Serology for infectious agents, microbiology and autoimmune blood work studies were negative. Computed tomography scan of the neck revealed small cervical lymphadenopathy and enlarged axillary lymphadenopathy. She underwent biopsy of a right axillary lymph node, which revealed Kikuchi lymphadenitis xanthomatous type. Skin biopsy showed unspecified perivascular lymphocytic infiltrate. On a three-month follow-up visit, she continued to be afebrile, had improved white cell blood count and had only small right axillary lymphadenopathy visible on ultrasound. Skin lesions had disappeared. Conclusion: Kikuchi-Fujimoto disease should be considered in the differential diagnosis when a young woman presents with fever and cervical lymphadenopathy. Despite its benign and self-limited nature, patients should be followed for a few years since recurrence is common and some patients may develop autoimmune disorders.

La maladie de Kikuchi-Fujimoto ; un diagnostic différentiel méconnu du lymphome chez l’adolescent

Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis, is a rare cause of lymphadenopathy in children. This benign disease can mimic lymphoma and misleads doctors. It was first described in Asia, where it occurred especially in young women. Recent publications show that it can also affect teenagers and young adults in Caucasian populations. The pathophysiology remains unknown. Three hypotheses have been raised for this disease: the role of viruses (in particular HHV-8), genetic predisposition (two alleles in HLA class II geneswere found more frequently in patients with Kikuchi disease), and an autoimmune cause because of the correlation with lupus erythematosus. Few cases have been reported in Europe so far. In this article, we report three cases of Kikuchi disease observed in less than 2 months in a single hospital in France. All three patients were teenagers who presented with lymphadenopathy, either isolated or combined with fever, weakness, and weight loss. In all of them, the hypermetabolic activity of the lymph node on the PET scanner misled us to suspect lymphoma. The diagnosis of Kikuchi disease was finally made, for all patients, after 2 weeks in the hospital based on lymph node biopsy. Based on this report, we highlight that early biopsy in presence of lymphadenopathy can avoid unnecessary extensive investigations. Moreover, in this rare disease, it is very surprising to come across three cases that are not family-related, in such a short period of time. This strengthens the hypothesis of the possible implication of an environmental factor in the pathophysiology of Kikuchi disease.

Kikuchi-Fujimoto disease, a rare cause of lymphadenopathy in Africa. Description of the first case in Senegal and review of the literature

IntroductionKikuchi-Fujimoto disease, or histiocytic necrotizing lymphadenitis, is a benign disease most commonly affecting adult females. It generally presents in the form of febrile cervical lymphadenopathy. This diagnosis must be considered in the setting of tropical medicine, dominated by a high prevalence of tuberculous lymphadenitis and haematological malignancies. Case reportThe authors report the case of a 33-year-old Senegalese woman who presented with subacute cervical lymphadenopathy associated with fever and laboratory signs of inflammation. Serological and tuberculosis screening tests were negative. Histopathological examination of a lymph node biopsy, following failure of nonspecific antibiotic therapy, concluded on a diagnosis of Kikuchi-Fujimoto disease. A favourable course was observed in response to corticosteroid therapy. ConclusionIn Sub-Saharan Africa, the association of polyadenitis and febrile syndrome, after excluding tuberculosis and lymphomas, must raise the suspicion of rare diseases such as Kikuchi-Fujimoto disease.

Castleman's Disease as a Rare Differential Diagnosis of Lymphadenopathy: 2 Case Reports and Review of Literature

ntroduction: Lymphadenopathy is a common complaint in children. It's defined as abnormality in size or character of lymph node. It may be localized or generalized. It may be part of a group of signs and symptoms or the sole finding and chief complaint. Several reasons can lead to Lymphadenopathy. Castleman's disease is a rare differential diagnosis of generalized or localized Lymphadenopathy. It's a rare lymph proliferative disorder that rarely occurs in children. Case presentation: We reported two cases, the first one was 13 years old female with multicenteric castleman's disease associated with constitutional manifestations and unusual hyaline vascular pathology successfully treated with monthly combination chemotherapy, the second one was 2.5 years old male with unicentric hyaline vascular castleman's disease with unusual young age at presentation successfully treated with surgical excision. Conclusion: Castleman's disease is a rare cause of Lymphadenopathy. Although, it's rare in children, it can occur at any age. Most of cases of multicenteric castleman's disease are plasma cell variant, yet hyaline vascular type may occur. Multicenteric castleman's disease can be successfully treated with monthly combination chemotherapy. Most cases of unicentric castleman's disease are hyaline vascular type. Although, surgical removal of unicentric type is usually sufficient to achieve complete remission, recurrence can still occur.

Kikuchis lymphadenitis: Is biopsy necessary in managing these patients

Kikuchis-Fujimoto's is a benign, self-limiting, cervical lymphadenitis, fine-needle aspiration cytology (FNAC) plays a very important role in the diagnosis and management of these patients. We present a case of 30-year-old female presenting with supraclavicular lymphadenopathy and fever, diagnosed as Kikuchis-Fujimoto's lymphadenitis on FNAC with no recurrence or development of Lupus lymphadenitis even after 1-year of follow-up. Although Kikuchis-Fujimoto's is a rare cause of lymphadenopathy, FNAC is an important tool in diagnosing this entity and differentiating from tubercular lymphadenitis and Lupus adenitis. Presence of cresentric nucleated histiocytes engulfed karryorrhectic debris and monocytoid cells aids in the diagnosing. A definitive diagnosis made on FNAC can avoid biopsy and unnecessary diagnostic and therapeutic interventions in these patients. However, a look out for recurrence and development of Lupus with regular followup and serological examination is imperative.

Kikuchi-Fujimoto disease: retrospective study of 91 cases and review of the literature.

Kikuchi-Fujimoto disease (KFD) is a rare cause of lymphadenopathy, most often cervical. It has been mainly described in Asia. There are few data available on this disease in Europe. We conducted this retrospective, observational, multicenter study to describe KFD in France and to determine the characteristics of severe forms of the disease and forms associated with systemic lupus erythematosus (SLE). We included 91 cases of KFD, diagnosed between January 1989 and January 2011 in 13 French hospital centers (median age, 30 ± 10.4 yr; 77% female). The ethnic origins of the patients were European (33%), Afro-Caribbean (32%), North African (15.4%), and Asian (13%). Eighteen patients had a history of systemic disease, including 11 with SLE. Lymph node involvement was cervical (90%), often in the context of polyadenopathy (52%), and it was associated with hepatomegaly and splenomegaly in 14.8% of cases. Deeper sites of involvement were noted in 18% of cases. Constitutional signs consisted mainly of fever (67%), asthenia (74.4%), and weight loss (51.2%). Other manifestations included skin rash (32.9%), arthromyalgia (34.1%), 2 cases of aseptic meningitis, and 3 cases of hemophagocytic lymphohistiocytosis. Biological signs included lymphocytopenia (63.8%) and increase of acute phase reactants (56.4%). Antinuclear antibodies (ANAs) and anti-DNA antibodies were present in 45.2% and 18% of the patients sampled, respectively. Concomitant viral infection was detected in 8 patients (8.8%). Systemic corticosteroids were prescribed in 32% of cases, hydroxychloroquine in 17.6%, and intravenous immunoglobulin in 3 patients. The disease course was always favorable. Recurrence was observed in 21% of cases. In the 33 patients with ANA at diagnosis, SLE was known in 11 patients, diagnosed concomitantly in 10 cases and in the year following diagnosis in 2 cases; 6 patients did not have SLE, and 4 patients were lost to follow-up (median follow-up, 19 mo; range, 3–39 mo). The presence of weight loss, arthralgia, skin lesions, and ANA was associated with the development of SLE (p < 0.05). Male sex and lymphopenia were associated with severe forms of KFD (p < 0.05). KFD can occur in all populations, irrespective of ethnic origin. Deep forms are common. An association with SLE should be investigated. A prospective study is required to determine the risk factors for the development of SLE.

Kikuchi–Fujimoto disease: A rare cause of lymphadenopathy and fever

Background: Kikuchi–Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, was originally described in young women and is a rare, benign and self-limited condition of unknown cause, usually characterized by cervical lymphadenopathy and fever. Less frequently, patients may present with lymph node involvement of other sites, weight loss, night sweats, rash, fatigue, joint pain, leukopenia and hepatosplenomegaly. The diagnosis of KFD is made by lymph node biopsy, in order to exclude more serious conditions such as lymphoma.

A rare cause of lymphadenopathy near the terminal ileum: immunoproliferative small intestinal disease

A rare cause of lymphadenopathy near the terminal ileum: immunoproliferative small intestinal disease

Inflammatory pseudotumor of lymph nodes presenting as pyrexia of unknown origin

Inflammatory pseudotumor (IPT) is an uncommon benign disorder characterized by proliferation of spindle cells, inflammatory cells, and small vessels. The IPT of lymph nodes is a rare cause of lymphadenopathy that usually affects one or two nodal groups. We describe a 27-year-old male presenting with generalized lymphadenopathy, hepatosplenomegaly and fever for 1 year. Histologic examination of lymph node revealed few remnant lymphoid aggregates with marked sclerosis and numerous anastomosing blood vessels in lymph node parenchyma. Interspersed in between the fibrotic bands was a polymorphic infiltrate composed of lymphocytes, plasma cells, eosinophils, and immunoblasts. Also, many histiocytes, multinucleated giant cells some being Langhan's type and at places forming ill-defined granulomas were observed. The presence of granulomas and Langhan's type of giant cells can lead to a misdiagnosis of tuberculosis as was also done in the present case. It is thus not only important to be aware of this rare benign pathologic entity while dealing with a case of lymphadenopathy, but also consider it in the differential diagnosis of granulomatous disease.

Lymphadenopathy due to amyloid deposition in non-Hodgkin’s lymphoma

Amyloidosis is a rare complication of non-Hodgkin's lymphoma. Most of the reported patients have had systemic amyloidosis and have died as a result of complications of this disease. The clinical cases of two patients with lymphoplasmacytic non-Hodgkin's lymphoma who presented with lymphadenopathy due to localised amyloid deposition are reviewed. Immunohistochemical studies were performed on the amyloid deposits and adjacent lymphoma. The amyloid deposits in both patients were derived from monoclonal light chains of the same isotype as those expressed by the lymphoma cells and were localised to areas adjacent to the lymphoma despite the presence of circulating light chains. Both patients had an indolent clinical course and treatment appeared to have little influence on the amyloid deposition. Non-Hodgkin's lymphoma may be associated with localised amyloidosis secondary to local production and deposition of amyloid from monoclonal light chains synthesised by the lymphoma cells. This is a rare cause of lymphadenopathy which does not respond to treatment of the underlying lymphoma.

Sinus histiocytosis with massive lymphadenopathy.

Sinus histiocytosis with massive lymphadenopathy is a well recognised, but rare cause of lymphadenopathy in the first decade of life. Three cases presenting with nodal disease are described. The eyelids were involved in one case. The clinical, laboratory, and biopsy findings are discussed and compared with previously reported cases.