Osteonecrosis of the Hip in Patients with Aplastic Anemia

The incidence and clinical and magnetic resonance imaging features of osteonecrosis of the hip were evaluated in patients with aplastic anemia. Two hundred and forty-one patients with aplastic anemia were examined using MR imaging of bone marrow during the five years from 1994 to 1998. Osteonecrosis of the hip was observed on MR imaging in nineteen (15 males and 4 females, mean age 35 yr) of the 241 patients. It was present in both hips in 14 patients, and there were five cases with unilateral occurrence, with a total of 33 involved hips. All except for five hips with associated bone marrow edema revealed increased fatty marrow conversion in the proximal femoral metaphysis. In nine patients, osteonecrosis was detected without any pain. Five patients already had osteonecrosis before any medication was administered. Twelve patients received antilymphocyte globulin, and seven patients received a low dose of steroids before the MR diagnosis of osteonecrosis. Osteonecrosis of the hip frequently develops in patients with aplastic anemia (7.9%), associated with fatty marrow conversion of the proximal femoral metaphysis.

A Clinical Observation on Leukemia Cutis / 대한피부과학회지

BACKGROUND: Leukemia cutis is readily recognized and documented by biopsy, in contrast. to leukemic involvement in more occult sites. Nine cases of leukemia cutis have been reported in the Korean literatures. However no collective clinical studies have been reported in Korea. OBJECTIVE: We evaluated the differences in patient age and sex, the clinical appearences and distributions of the skin lesions, interval between diagnosis of systemic leukemia and skin involvement, clinical course, and prognosis according to the type of leukemias. METHODS: We carried out a retvospective study of 22 cases of leukemia cutis. Clinical information was obtained from the records of of 22 patients diagnosed at St. Mary's Hospital from 1988 to 1995. All the included cases were well evaluated for their clinical and histopathologic findings. RESULTS: 1. Among 22 patients with leukemia cutis, male patients outnumbered female by 2 to 1 and the mean age was 25.8 years. 2. The clinical appearance of leukemia cutis includes papules, macules, nodules, plaques in all types of leukemia. Ulcerative lesions and vesicles were seen infrequently in leukemia cutis. Leukemia cutis often involved saultiple location of the skin, with no specific predilection of the site. There were no differences in distribution of lesions depending on the types of systemic leukemia. 3. In 68% of the patients with leukemia cutis, the skin lesions developed after the systemic leukemia was diagnosed, and 14% of patients had concomitant, involvement. 18% of patients had skin lesions preceding the diagnosis of systemic leukemia, howevere cytochemical and cytomorphologic studies of bone marrow and peripheral blood smear were not employed at the time of the skin biopsy. 4. Fourteen of 22 patients(64%) did not achieve a complete remission following the diagnosis of leukemia cutis and two of 14 patients without having complete remission could achieve complete remissions with proper anticancer therapy after the diagnosis of leukemia cutis. Total eight patients(36%) achieved a complete remission, then they had a relapse of leukemia in the skin, without having had any skin involvement at the time of the diagnosis of leukemia. 5. Seventeen of 22 patients(77% ) who were being followed up in our series died after leukemia cutis was diagnosed. The mean intervals between diagnosis of leukemia cutis and death was 3.8 months and they died mostly within 1 year. CONCLUSION: The presence of leukemic infiltration in the skin may help the clinician suspect the early diagnosis and relapse of systemic leukemia. It appears that leukemia cutis is associated with a grave prognosis.

Effect of Granulocyte-Macrophage Colony-Stimulating Factor (GM-CSF) on Neutropenia Occuring during Radiotherapy / 대한치료방사선과학회지

PURPOSE: To assess the efficacy of recombinant human granulocyte-macrophage colony-stimulatin g factor(GM-CSF) in the neutropenia by radiotherapy. MATERIALS AND METHODS: Eleven patients with various solid tumor were treated with a daily subcutaneous dose of GM-CSF(3-7 microgram/kg) for 5 days during the radiotherapy. Before and during the course of the study all the patients were monitored by the recording of physical examination, the complete blood count with differential and reticulocyte count and liver function test. Eight patients received patients received prior or concurrent chemotherapy. RESULTS: In 10 patients, the neutrophilic nadir was significantly elevated and the length of time that patients had a neutrophil count below 103/mm3, a threshold known to be critical to acquiring infective complications was shortened following GM-CSF injection. A significant rise (two fold or greater) of neutrophil count was seen in 10 of 11 patients. In most patients, discountinuation of GM-CSF resulted in a prompt return of granulocyte counts toward baseline. However the neutrophil count remained elevated over 103/mm3 during radiation therapy, and radiotherapy delays were avoided. Other peripheral blood components including monocytes and platelets also increased after GM-CSF treatment. No siginificant toxicity was encountered with subcutaneous GM-CSF treatment. CONCLUSION: GM-CSF was well tolerated by subcutaneous route and induced improvement in the neutropenia caused by radiotherapy.

Richer's Syndrome: Report of a case

Richer's syndrome is a development of a high grade malignant lymphoma in a patient with preexisting chronic 1ymphocytic leukemia, small lymphocytic lymphoma or Waldenstrom's macroglobulinemia. A rare case of Richer's syndrome arising in the spleen of a 35-year-old-man was studied by morphology, immunohistochemistry and gene rearrangement study. He has had weight loss and night sweat for last 6 months. Hepatosplenomegaly and abdominal lymphadenopathy were noted on CT scanning. Especially an ovoid radiolucent mass was found within the image of splenomegaly. Lymph nodes and liver biopsy, bone marrow aspiration and splenectomy were done. In the lymph nodes, liver and bone marrow, well differentiated small lymphocytic infiltrations were found but, in the spleen, pleomorphic, large cells with occasional multinucleated giant cells formed a nodular mass surrounded by diffuse, extensive infiltration of small well differentiated lymphocytes. The two distinctive areas in the spleen had positive staining for B-cell marker (HLA-DR and L26), negative staining for T-cell marker (UCLH1), and positive staining for IgM heavy chain and kappa light chain by immuohistochemical study. so this case was diagnosed as a diffuse large cell 1ymphoma transformed from small lymphocytic lymphoma. We made an another effort to clarify their clonality. Gene rearrangement method usingcomplementarity.determining region 3(CDR3) of immunoglobulin heavy chain (IgH) gene and T-cell receptor gamma (TCRgamma) gene by polymerase chain reaction (PCR) technique was done. The two lymphomas in the spleen demonstrated the same rearrangement pattern in both IgH and TCRgamma gene. We think these findings strongly suggest that the large cell lymphoma has the same clonality with that of the small lymphocytic lymphoma.

A Case of Acute Graft-versus-Host Disease / 대한피부과학회지

We report a case of acute graft-versus-host disease, which developed after bone marrow transplantation because of acute myelocytic leukemia in a 39-year old male, The pruritic, erythematous maculopapular eruptions began to developed on the perioral regions, and spreaded the face, the oral mucosa, both hands, and buttocks at the twenty fourth day after bone marrow transplanta.tion. The eruptions were confluent to form erythematous patches. Iistopathological findings show parakeratosis, lymphoid cell exocytosis, and papillary edema, lymphohistiocytic infiltration, and melanophage in the upper dermis, and basal vacuolation. He was treated systemically by methylprednisolone, and antilymphocytic globulin, and tapically by emollients and steroids.