Extramedullary Hematopoiesis: An Unusual Finding in Angiolymphoid Hyperplasia With Eosinophilia.

Extramedullary hematopoiesis is the process of blood cell synthesis occurring outside the medulla of the bone marrow. During fetal development, extramedullary hematopoiesis is considered physiological; however, it is considered pathologic when occurring outside the neonatal period. Angiolymphoid hyperplasia with eosinophilia (ALHE) describes a lesion characterized histologically by plump endothelial cells associated with a mixed inflammatory infiltrate of lymphocytes, plasma cells, mast cells, and eosinophils. This report describes an adolescent boy with an isolated preauricular facial lesion. After excision, histopathology confirmed a diagnosis of ALHE with the additional finding of trilineage extramedullary hematopoiesis. To the authors' knowledge, this is the first reported case of extramedullary hematopoiesis in ALHE.

Kimura's disease affecting the axillary lymph nodes: a case report.

BACKGROUND: Kimura's disease (KD; eosinophilic granuloma of soft tissue) is an inflammatory granulomatous disorder of unknown cause with eosinophilic infiltration that occurs mainly in soft tissue. KD occurs mainly in the head and neck, but development in the axillary region is very rare. CASE PRESENTATION: A 74-year-old Japanese woman was evaluated for a mass that she noted in the left axillary region. On physical examination, there was a palpable, thumb-sized, hard, elastic, freely movable mass in the left axilla. Blood tests showed elevated soluble interleukin-2 receptor (sIL-2R), normal serum immunoglobulin (Ig) G4, and elevated serum IgE. Ultrasonography of the left axilla showed multiple lymph nodes (LNs) with irregular margins in which central hyperechogenicity was lost. A systemic search by computed tomography (CT) showed no systemic lymphadenopathy or other mass-like lesions suspicious for a primary tumour other than in the left axillary LNs. Biopsy of an excised LN was performed under local anaesthesia for a definitive diagnosis. Histopathology showed various-sized lymphoid follicles, large nodular lesions with an enlarged mantle zone, multiple various-sized germinal centres in single nodules, and eosinophilic infiltration between the nodes. Immunohistochemical (IHC) staining of the germinal centres was positive for cluster of differentiation (CD) 10, positive for B-cell lymphoma (bcl)-6, and negative for bcl-2. These findings led to a diagnosis of KD. Ultrasound after 3 months of follow-up showed disappearance of the axillary lymphadenopathy. CONCLUSIONS: A very rare case of KD in the axillary LNs was described. KD has the potential to occur in any region.

Clinicopathological features and prognosis of Kimura's disease with renal involvement in Chinese patients.

AIMS: Kimura's disease (KD) with renal involvement is a rare disease. Optimal treatments are still not well established. It is necessary to analyze clinicopathological features, treatment responses, and prognosis for improving KD diagnosis and treatment. MATERIALS AND METHODS: Clinicopathological data, treatment responses, and prognosis were collected and analyzed retrospectively. RESULTS: The patients consisted of 27 males and 2 females, with an average age of 35.5 ± 15.1 (13 - 61) years. 27 exhibited proteinuria ranging from 0.730 to 14.1 g/24 h (5.98 ± 3.40 g/24 h). Hypertension, renal insufficiency (serum creatinine (Scr) > 1.24 mg/dL), and microhematuria occurred in 4 (13.8%), 11 (37.9%), and 13 (44.8%) cases, respectively. Light microscopy (LM) identified mesangium proliferation, minimal change, focal and segmental glomerulosclerosis (FSGS), membranous glomerulonephritis, membranoproliferative glomerulonephritis (MPGN), and acute tubular necrosis in 14, 8, 3, 2, 1, and 1 cases, respectively. All were treated with Tripterygium wilfordii (TW), prednisone, leflunomide (LEF), tacrolimus (FK506), myophenolate mofetil (MMF), or renin-angiotensin system blockers (RASI). 26 patients were followed up for 1.60 - 108.7 months (39.6 ± 28.7). After treatments, urinary red blood cells (RBC) decreased in all. The amount of 24-hour urinary protein (24-hUPE) decreased in 24 patients. 22 reached complete remission (CR), 4 partial remissions (PR). The patients who did not relapse were younger than those who relapsed. CONCLUSIONS: KD with renal involvement occurs predominantly among 35 - 50 year old Chinese patients with male predilection. The most common features are proteinuria, hypertension, micro hematuria with minimal change, and mesangial proliferative glomerulonephritis. Most were responsive to treatment, but could relapse. Gender, age, and hypertension are associated with KD recurrence. The prognosis is good mostly.

Variante linfoide del síndrome hipereosinófilo / Lymphocytic variant of the hypereosinophilic syndrome

Fundamento: El síndrome hipereosinófilo (SHE) es un grupo heterogéneo de procesos raros caracterizados por un aumento prominente y sostenido de eosinófilos en sangre y daño orgánico. Se deben excluir otras causas de hipereosinofilia. Avances recientes en biología molecular y citogenética han permitido caracterizar diferentes subtipos. Se describe un caso de la variedad linfoide. Paciente: Varón filipino de 46 años que consulta por lesiones cutáneas, fiebre, conjuntivitis, adenopatías y eosinofilia prominente. Resultados: El estudio de poblaciones linfocitarias en sangre periférica revela una población aberrante de linfocitos T (LT) CD3-CD4+ productora de interleucina 5 (IL-5). Se confirmó la clonalidad del TCR mediante PCR. Conclusiones: La variedad linfoide del SHE está caracterizada por una expansión clonal de LT no maligna que produce IL-5, de comportamiento indolente, pero que puede evolucionar a linfoma T periférico. Se describe el primer caso de variedad linfoide publicada en nuestro país y se revisan las características de esta variedad (AU)

Objectives: Hypereosinophilic syndromes (HSS) are a rare group of heterogeneous disorders characterized by prominent and persistent eosinophilia and organ dysfunction. Secondary causes of eosinophilia must be excluded. Recent advances in molecular biology and cytogenetics have permitted the characterization of different subsets of hypereosinophilic syndrome. We describe a patient with the lymphocytic variant. Patient: A 46-year old male Philippine patient presented skin lesions, fever, red eyes, enlarged lymph nodes and marked eosinophilia. Results: Lymphocytic phenotyping by flow cytometry analysis was performed on peripheral blood and an aberrant population of T lymphocytes CD3-CD4+ producing interleukin 5 was found. TCR gene rearrangement using PCR amplification confirmed T cell clonality. Conclusions: The lymphocytic variant of the hypereosinophilic syndrome is a primitive lymphocytic disorder characterized by a non-malignant T cell population expansion producing eosinophilopoietic cytokines, with an indolent clinical course but that can transform into a peripheral T lymphoma. We report the first case of such a variant published in our country and review the characteristics of this variety (AU)

Kimura's disease in children: a 9 years prospective study.

UNLABELLED: Kimura's disease is a rare form of chronic inflammatory disorder involving subcutaneous tissue, predominantly in the head and neck region and frequently associated with lymphadenopathy and/or salivary gland enlargement. The nodular lesions are deep seated in subcutaneous tissue and clinically may mimic a neoplasm. Hence head and neck surgeons need to be aware of clinical presentation of Kimura's disease. OBJECTIVES: To study the clinical presentations, management and complications of Kimura's disease in pediatric age group. STUDY DESIGN: Prospective study. MATERIALS AND METHODS: The duration of study was 9 years (January 1998 to December 2006), comprising of 18 patients. Only histopathologically proven cases were included in this study. Blood eosinophil count and serum IgE estimation were done in all these patients. All the patients underwent fine needle aspiration cytology study. In 15 patients excision biopsy was done and resected specimen was sent for histopathological examination. In three cases, only biopsy was done to confirm the diagnosis. RESULTS: All the patients presented with painless swelling in the head and neck region. Post-auricular region was the commonest site (50%). Sixteen patients (88.8%) had blood eosinophilia and in 15 patients (83.3%) serum IgE level was elevated. Fifteen cases were treated by surgery and three cases were treated with steroids. Out of 18 cases, 15 cases were symptom free at the end of 1 year. Among 15 patients who underwent surgery, only one had recurrence (6.6 %). Out of three patients who were treated with corticosteroids, two came back with recurrence (66.6%). In our study, totally three patients had recurrence (16.6%). One patient had nephrotic syndrome (5.5%). CONCLUSION: Post-auricular region is the commonest site of involvement. Incidence is more common in the second decade of life. Recurrence rate is more with steroid therapy. Surgery is the best modality of treatment. The only systemic complication is nephrotic syndrome.

Kimura's disease in Malay patients.

Kimura's Disease (KD) is an uncommon, chronic inflammatory disorder of unknown etiology which is endemic in Orientals. It is characterized by painless, large solitary or multiple nodules in subcutis of head and neck region or the major salivary glands, associated with regional lymphadenopathy, blood eosinophilia and elevated IgE levels. Its treatment ranging from conservative observation in asymptomatic patient to surgical resection of the mass, corticotherapy and irradiation therapy for the symptomatic ones.

Nódulo asintomático en la frente / Asymptomatic nodule on forehead

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Recurrent Kimura's disease: excellent response to cyclosporine.

Kimura's disease is a chronic inflammatory disorder involving the skin, subcutaneous tissues and lymph nodes, predominantly in the head and neck region. Though surgery, intralesional or systemic steroids or radiation therapy have been the mainstay of treatment recurrence is a common problem. On the basis of occasional case report of Kimura's disease responding to cyclosporine, we attempted oral cyclosporine in our patient with dramatic improvement.

Sleep apnea due to Kimura's disease of the larynx. Report of a case.

Kimura's disease is a benign chronic granulomatous disease which presents as a subcutaneous swelling in the head and neck area. The histopathological feature consists of granuloma-forming lymphoid follicles with eosinophil infiltration. Kimura's disease of the larynx is very rare, and only a few cases have been reported. We report a 14-year-old boy who presented with sleep apnea. Laryngological study revealed a submucosal swelling of the bilateral false vocal cord. Histopathological examination showed lymphoid hyperplasia with marked infiltration of eosinophils, which was diagnosed as Kimura's disease. After laser surgery, he had recurrent swelling of the false cord. The patient was given 30 mg of prednisolone, which was gradually tapered. The laryngeal swelling resolved, and the sleep apnea immediately improved after the treatment. However, a low dose of prednisolone was necessary to maintain the remission. Oral administration of pranlukast successfully supported the tapering of prednisolone.

A case of pathophysiologic study in Kimura's disease: measurement of cytokines and surface analysis of eosinophils.

BACKGROUND: Kimura's disease is a rare but distinctive eosinophilic inflammatory disorder of unknown etiology; few reported case studies have focused on the immunopathologic background of this unique disease. OBJECTIVE: To define better the immunopathogenetic features of Kimura's disease, we attempted to quantitatively analyze values of cytokines and soluble interleukin-2 receptor (sIL-2R) in peripheral blood (PB), as well as perform surface immunophenotypic analysis of eosinophils from a Japanese patient with chronic relapsing Kimura's disease. RESULTS: Granulocyte macrophage-colony stimulating factor (GM-CSF), tumor necrosis factor-alpha (TNF-alpha) and sIL-2R were elevated, and newly expressed antigens on eosinophils CD4, CD25, and HLA-DR were found to be involved in the pathophysiology of this disorder. CONCLUSIONS: Kimura's disease may be a disease in which activated lymphocytes release cytokines, and these released cytokines, such as GM-CSF and TNF-alpha cause eosinophil activation. These processes may be related to the pathogenesis of this disorder.

Hiperplasia angiolinfoide con eosinofilia: presentación de dos casos y diferenciación con la enfermedad de Kimura / Angiolymphoid hyperplasia with eosinophilia: report of two cases and differentiation with kimura's disease

Se presentan dos casos de hiperplasia angiolinfoide con eosinofilia. Un hombre de 64 años de edad con una lesión nodular en el canto interno del ojo y una mujer de 35 años con lesiones pápulo-nodulares en región auricular. Se discuten algunas diferencias con la enfermedad de Kimura