Child ; Histiocytosis, Sinus ; diagnosis ; drug therapy ; Humans ; Lymphatic Diseases ; diagnosis ; drug therapy ; Male

Leukemic reticuloendotheliosis (L.R.E.) was first described as a clinical and pathological entity by Ewald in 1923. LRE is a rare neoplastic disease of the hematopoietic system that is characterized cliniclly by chronic course with an insidious onset. marked splenomegaly with absence of substantial lymphadenopathy and predominant in male and characterized histologically by the presence of circulating abnormal mononuclear cells with many cytoplasmic projections, which have been refered to ??airy cells frequently. Splenectomy appeared to be the most beneficial treatment at present for those patients with massive splenomegaly and hypersplenism and chemotherapy is of little benefit in the treatment in LRE. Recently, the authors had the opportunity to observe a two year and eight old male child at this hospital whose clinical course and pathologid features were consistent with LRE. Splenectomy is performed and discharged with relatively good conditions.
Child ; Cytoplasm ; Drug Therapy ; Hematopoietic System ; Humans ; Hypersplenism ; Leukemia, Hairy Cell* ; Lymphatic Diseases ; Male ; Splenectomy ; Splenomegaly

Adult ; Humans ; Hypereosinophilic Syndrome ; complications ; drug therapy ; Lymphatic Diseases ; diagnosis ; etiology ; pathology ; Male ; Treatment Outcome

We report here a case of angioimmunoblastic lyinphadenopathy with dysprote inemia(AILD) in a 65-year-old male associated with skin nodules on the face. Physical examina.tion showed generalized lymphadenopathy, hepatosplenomegaly, erythematous maculopapular eruptions on the trunk and extremities, and er the matous nodules on the face. Lahoratory findings showed hemolytic anernia, arid polyclonal gammopathy. Histopathological findings of lymph node revealed typical features of AILD. I-iistopathological findings of maculopapules revealed lymphohistiocytic infiltra tion and extravasated erythrocytes around the blocked vessels, whereas histological picture of nodules on the face mirrorred that of the lymph node. After taking systemic chemotherapy, his general condition and skin lesions were improved.
Aged ; Drug Therapy ; Erythrocytes ; Extremities ; Humans ; Immunoblastic Lymphadenopathy* ; Lymph Nodes ; Lymphatic Diseases ; Male ; Skin*

Rosai-Dorfman disease (RDD) is a rare and benign self-limited disorder with pathologic feature of the lymph node sinuses expanded by a proliferation of distinctive histiocytes. The most often involving site is bilateral cervical lymphadenopathy, followed by the skin and soft tissue. Treatment options, including steroid, chemotherapy, radiotherapy and debulking surgery depend on the symptoms or the extent and localization of the lesions. We encountered a very rare case of RDD at the skin lesions, particularly combined with aortic vasculitis, arrhythmia, and valvular heart disease.
Arrhythmias, Cardiac* ; Drug Therapy ; Heart Valve Diseases* ; Histiocytes ; Histiocytosis, Sinus* ; Lymph Nodes ; Lymphatic Diseases ; Methotrexate ; Prednisolone ; Radiotherapy ; Skin ; Vasculitis*

A 32-year-old man visited our clinic with complaints of left epiphora and neck masses. A computed tomographic scan demonstrated a well-demarcated homogeneous mass in left lacrimal sac area and a polypoid mass in ethmoidosphenoidal sinuses, and showed multiple lymphadenopathy in neck. Dacryocystectomy and ethmoidosphenoidectomy were performed. Pathologic diagnosis by immunohistochemical study and electron microscopic examination was alveolar type rhabdomyosarcoma. Subsequently, chemotherapy and radiotherapy were done. There was no evidence of recurrence of the tumor in lacrimal sac and paranasal sinus throughout 9 months of follow-up period. The masses in the neck nearly disappeared. Rhabdomyosarcoma in one's thirties is extremely rare and to our knowledge, this patient represents the first case reported to have developed such a tumor in the lacrimal sac. Although rhabdomyosarcoma is very uncommon, there is a rationale for including it in differential diagnosis of a rapidly growing tumor in the lacrimal sac.
Adult* ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Follow-Up Studies ; Humans ; Lacrimal Apparatus Diseases ; Lymphatic Diseases ; Neck ; Radiotherapy ; Recurrence ; Rhabdomyosarcoma*

Macrophage activation syndrome (MAS) is one of the serious complications of juvenile rheumatoid arthritis (JRA) and recently, cyclosporine A has been found to be effective in patients with corticosteroid-resistant MAS. A 29-yr-old male was admitted with high fever and jaundice for one month. He was diagnosed as juvenile arthritis 16 yr ago. Physical and laboratory results showed hepatosplenomegaly, high fever, pancytopenia and impaired liver and renal function tests, elevated triglyceride and serum ferritin levels. Bone marrow biopsy showed hyperplasia of histiocytes with active hemophagocytosis. He was diagnosed as MAS associated with juvenile rheumatoid arthritis and managed with high-dose corticosteroids initially, but clinical symptoms and laboratory findings did not improve immediately. Finally, he completely recovered after treatment with cyclosporine A (3 mg/kg/day).
Treatment Outcome ; Pancytopenia/*drug therapy ; Male ; *Macrophage Activation ; Lymphatic Diseases/*drug therapy ; Immunosuppressive Agents/therapeutic use ; Humans ; Cyclosporine/*therapeutic use ; Arthritis, Juvenile Rheumatoid/*drug therapy ; Adult

We report a case of an erythrodermic form of mycosis fungoides in a 68-year-old male, who showed generalized erythroderma with scales for 3 months. Lymphadenopathies in the inguinal and neck areas were present. Histopathological findings showed epidermotropism, perivascular atypical lymphocyte infilteration in the upper dermis, and dermatopathic lymphadenopathy in the inguinal lymph node. T cell markers were positively stained in the immunohistochemical study. These clinicopathological features were consistent with an erythrodermic form of mycosis fungoides(TNM IIl). The patient was treated with PUVA therapy and low dose chemotherapy.
Aged ; Dermatitis, Exfoliative ; Dermis ; Drug Therapy ; Humans ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Male ; Mycosis Fungoides* ; Neck ; PUVA Therapy ; Weights and Measures

A 37-year-old woman was admitted to our hospital because of cervical and axillary lymphadenopathy that developed after delivery. An axillary lymph node biopsy revealed metastatic adenocarcinoma. Immunohistochemical staining indicated that the tumor cells expressed c-ErbB-2, but were negative for the estrogen and progesterone receptors. No definite evidence of breast cancer was detected. The patient underwent chemotherapy for suspected metastatic breast cancer. She complained of swelling in the left breast 22 months later, and a biopsy showed invasive ductal carcinoma. Here, we report a case of hormone receptor-negative occult breast cancer in a patient with cervical and axillary lymphadenopathy presenting as a cancer with an unknown primary site.
Adenocarcinoma ; Adult ; Biopsy ; Breast Neoplasms* ; Breast* ; Carcinoma, Ductal ; Drug Therapy ; Estrogens ; Female ; Humans ; Lymph Nodes ; Lymphatic Diseases ; Receptors, Progesterone

We herein report a case of Richters syndrome(RS) that was discovered following an investigation of a skin lesion. RS represents an acute transformation of chronic lymphocytic leukemia(CLL) to highly malignant large cell lymphoma such as diffuse large B-cell lymphoma(DLBCL). RS is characterixed by abrupt B-symptoms, rapidly progressive lymphadenopathy, hepatosplenomegaly, frequent extranodal involvement, hypercalcemia and an unfavorable clinical course. The patient is a 31-year-old male with skin lesions on his face. He had been previously diagnosed with CLL. At his presentation, CLL and DLBCL manifestly co-existed. Our multistage analysis suggested that DLBCL and CLL might arise from a different B-cell clone. He showed a poor clinical response to systemic chemotherapy.
Adult ; B-Lymphocytes ; Clone Cells ; Drug Therapy ; Humans ; Hypercalcemia ; Leukemia, Lymphocytic, Chronic, B-Cell* ; Lymphatic Diseases ; Lymphoma* ; Male ; Skin