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

Herein we report a 51-year-old male with angioimmunoblastic lymphadenopathy who had developed rnaculopapular eruptions after the administration of crystalline penicillin, acetylsalicylic acid and sulpyrine. Skin biopsy showed mild lyrnphohistiocytic infiltration and extravasated RBCs around the blood vessels in the upper dermis. He had run a progressively downhill course in spite of vigorous chemotherapy and ultimately died.
Aspirin ; Biopsy ; Blood Vessels ; Crystallins ; Dermis ; Dipyrone ; Drug Therapy ; Humans ; Immunoblastic Lymphadenopathy ; Lymphatic Diseases* ; Male ; Middle Aged ; Penicillins ; Skin

To see the usefulness of CT in evaluation of response to treatment in patients with mediastinal tuberculous lymphadenitis (MTL), we analyzed the initial CT patterns and follow-up CTs or serial plain radiographs during 18 months antituberculous chemotheraphy in 58 consecutive patients. CT patterns of MTL at the beginning of treatment were categorized into solid type (n=8), low density with peripheral rim enhancement type (n=36), extranodal extension type (n=9) and calcified type (n=5). According to the response to treatment, each patients was categorized into prompt response group (response within the first three months and no residual lymph node after one year), slow response group (response after three months but residual lesion after one year), no response group (no change in size during 18 months treatment) and temporal increase group (temporal increase in size during the treatment but ultimately improved with chemotheraphy). Among 29 cases of prompt response group, 20 cases had large low-density areas, 6 cases had diffuse or extensive node involvement with extranodal extension or tracheal compression. Two patients with acquired immune derfciency syndrome belonged to this group. Fifteen cases of slow response group had low-density nedes in 7 patients and small solid or extranodal-extension nodes in 6 patients. They were associated with disseminated pulmonary tuberculosis, generalized lymphadenopathy or other organ(pericardium, brain, bone or abdomen) involvement. In 5 cases of no response group, there were 3 cases of calcified node and 2 cases of small solid nodes. Nine cases of temporal increase group included low density type of MTL only. And they were associated with disseminated pulmonary of endobronchial tuberculosis. In conclusion, response to antituberculous chemotherapy could be predicted in patients with MTL on the basis of CT findings before treatment.
Brain ; Drug Therapy ; Follow-Up Studies ; Humans ; Lymph Nodes ; Lymphatic Diseases ; Tuberculosis ; Tuberculosis, Lymph Node* ; Tuberculosis, Pulmonary