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

Splenic irradiation in chronic myelogenous leukemia is reserved for patients who have painful splenomegaly despite chemotherapy and/or inoperable splenomegaly because of huge size. The role of splenic irradiation is diminution of painful splenomegaly and indirect effect of splenic irradiation on unirradiated hematopoietic and lymphoreticular tissue such as reduction of leukocyte count and increase of hemoglobin level. We report on a useful clinical method for splenic irradiation in chronic myelogenous leukemia. We have used sonography as the tool of simulation. The portal size using modified method is smaller than the field size of conventional simulation, and so this method suggests that useful to irradiation of huge splenomegaly, effective shielding of critical organ and the downfall of complication during irradiation of spleen.
Drug Therapy ; Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukocyte Count ; Spleen ; Splenomegaly

PURPOSE: This study investigated how adding Korean red ginseng extract (KRG) to folinic acid, fluorouracil and oxaliplatin (FOLFOX) chemotherapy affected the rate of splenomegaly in colon cancer. METHODS: This retrospective study analyzed 42 patients who were randomly assigned to receive a FOLFOX regimen with or without KRG. Spleen volume change was assessed by computed tomography scans measured before surgery (presurgery volume) and 3 weeks after cessation of the 12th cycle of FOLFOX (postchemotherapy volume). RESULTS: All patients showed increased spleen volume. No difference was observed in median presurgery and postchemotherapy volume between the KRG and control groups. However, a ratio defined as postchemotherapy volume divided by presurgery volume was significantly lower in the KRG group than the control group (median, 1.38 [range, 1.0–2.8] in KRG group vs. median, 1.89 [range, 1.1–3.0] in control group, P = 0.028). When splenomegaly was defined as a >61% increase in spleen volume, the rate of splenomegaly was significantly lower in the KRG group than the control group (28.6% vs. 61.9%, P = 0.03). KRG consumption was inversely associated with developing splenomegaly in multivariate analysis. CONCLUSION: Adding KRG during FOLFOX chemotherapy for colon cancer might protect against oxaliplatin-induced splenomegaly. The protective effect of Korean red ginseng should be investigated with further research.
Colon* ; Colonic Neoplasms* ; Drug Therapy ; Fluorouracil ; Humans ; Leucovorin ; Multivariate Analysis ; Panax* ; Retrospective Studies ; Spleen ; Splenomegaly*

Primary myelofibrosis (PMF) is easily confused with cirrhosis, due to its main clinical manifestations of splenomegaly and the blood cytopenia. This review focuses on clinical studies to identify primary myelofibrosis and cirrhosis related portal hypertension, to analyze the differences between the two diseases, in order to distinguish PMF and cirrhosis from the pathogenesis, clinical manifestations, laboratory examinations and treatment principles, and simultaneously improve clinicians' understanding of PMF, which is a reference for exploring the early screening or diagnostic indicators of PMF, also provides a clinical basis for the application of new targeted drugs such as ruxolitinib.
Humans ; Primary Myelofibrosis/drug therapy* ; Hypertension, Portal/complications* ; Liver Cirrhosis/pathology* ; Splenomegaly/pathology* ; Anemia

Lymphomatoid granulomatosis (LG) is an angiocentric lymphoproliferative disease. It usually involves lung, skin, and central nervous system, but splenomegaly and pancytopenia are the rare manifestations of the disease. We report a 15-year-old boy presented with fever, dry cough and dyspnea from two months ago, after admission patient had nodular lesions on the left leg and hepatosplenomegaly. Then he manifested neurologic signs such as seizure, aphasia and right-sided hemiplegia. Chest X-ray and CT scan revealed bilateral pulmonary nodules predominantly in lower lobes and peripheral lung fields. Laboratory exams showed pancytopenia. Skin biopsy was done, and histopathological examination and immunohistochemistry evaluation confirmed lymphomatoid granulomatosis. He was treated with steroid and cyclophosphamide but succumbed by neurologic involvement.
Adolescent ; Humans ; Lymphomatoid Granulomatosis ; diagnosis ; diagnostic imaging ; drug therapy ; Male ; Pancytopenia ; diagnosis ; diagnostic imaging ; drug therapy ; Radiography ; Splenomegaly ; diagnosis ; diagnostic imaging ; drug therapy

Multiple lymphomatous polyposis(MLP) is an interesting clinical entity of non-Hodgkin's lymphoma(NHL) and is defined as B-cell lymphoma characterized by the presence of multiple lymphomatous polyps along the gastrointestinal tract. Recently MLP has been considered as a variant form of mantle cell lymphoma(MCL). The median survival of patients with MCL is only 3 years, and none of the available conventional chemotherapy regimens appears curative. Encouraging results have been reported with high dose chemotherapy with autoSCT and alloSCT for its treatment. We introduce 4 cases of MLP diagnosed as MCL by morphologic and immunologic method. The common clinical findings of these cases were splenomegaly (4/4), multiple intraabdominal lymphadenopathy (4/4), and advanced stage (3/4) at presentation. The overall remission duration was relatively short (5-27 months) and three of four cases relapsed after conventional chemotherapy or autologous stem cell transplant. Our report suggests that MCL presented as MLP is a high risk subgroup of NHL and more aggressive approach may be needed for cure.
Drug Therapy ; Gastrointestinal Tract ; Humans ; Lymphatic Diseases ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, Mantle-Cell* ; Polyps ; Splenomegaly ; Stem Cells

Radiation therapy was the treatment of choice for CML in the past, in the form of Sl or radioactive phosphorus. Its use has been replaced to a large extent by various chemotherapeutic agents. Recently Sl in CML has been used, both to relieve painful splenomegaly and to take advantage of an indirect effect of Sl on unirradiated bone marrow. We have treated 15 CML cases who had a huge spleen during chemotherapy or even after chemotherapy by 6 MV linear accelerator during the past two years at the Division of Radiation Therapy, Kang Nam St. Mar's Hospital, Catholic College. Response to Sl has been rated according to the scoring system of Roger W. Byhardt, et al. which evaluated the splenic and hematologic response as well as the response of disease-elated systems. According to this scoring system, most patients demonstrated a significant relief of splenomegaly along with improvement of hemogram. And we observed the change of Karnofsky Performance Status after Sl, and survival after a confirmative diagnosis and Sl.
Bone Marrow ; Diagnosis ; Drug Therapy ; Humans ; Karnofsky Performance Status ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive* ; Particle Accelerators ; Phosphorus ; Spleen ; Splenomegaly

Splenic marginal zone lymphoma (SMZL) is a rare B-cell neoplasm characterized by massive splenomegaly, moderate lymphocytosis, bone marrow intrasinusoidal involvement of lymphocytes and a relatively indolent course. We report a case of SMZL diagnosed by bone marrow studies using immunophenotyping and immunohistochemical stain, and confirmed by splenectomy. The patient was a 61-year old male, who showed mild lymphocytosis in peripheral blood and bone marrow aspirates. Immunophenotyping of bone marrow aspirates showed lymphocytes positive for CD19, CD20, CD22 (dim), CD23 (dim) and negativie for CD5 and CD10. The immunohistochemistry of bone marrow and spleen also showed lymphocytes positive for CD20 and negative at for cyclin D1. Now he is being treated for chronic obstructive pulmonary disease and will receive chemotherapy.
B-Lymphocytes ; Bone Marrow ; Cyclin D1 ; Drug Therapy ; Humans ; Immunohistochemistry ; Immunophenotyping ; Lymphocytes ; Lymphocytosis ; Lymphoma* ; Male ; Pulmonary Disease, Chronic Obstructive ; Spleen ; Splenectomy ; Splenomegaly

PURPOSE: In this study, we analyzed clinical manifestations of and ways to manage malaria. METHODS: The medical records of the patients confirmed as malaria by using peripheral blood smear at Ui jungbu St. Mary's hospital from April 1997 to November 2001 were reviewed retrospectively. RESULTS: Of the 170 patients, there were 111 males and 59 females, and their mean ages were 32.3+/-14.5, 34.9+/-18.1 years, respectively. Malaria occurred throughout the year and peaked during the sixth to ninth months (84.1%). All cases were vivax malaria. All 170 patients had fever, but tertian fever was seen in only 81 (48%) patients. Laboratory abnormalities were hemoglobin below 12 g/dL in 70 (41.2%) patients, WBC below 4000/mm3 in 49 (28.9%), platelet count below 120,000/mm3 in 142(83.5%). Of the 92 patients who underwent ultrasonography of the abdomen, splenomegaly was seen in 81 (89%)patients. The time from onset of symptoms to admission ranged from 2 to 30 days with a mean 8.1+/-5.5 days. All patients responded promptly to drug therapy. One patient developed recurrent malaria 120 days after the first attack. Another patient experienced multifocal splenic infarction. CONCLUSION: All patients with malaria had fever and chills. The most common laboratory findings were thrombocytopenia and anemia. All patients responded promptly to drug therapy. As cases of malaria in Korea are increasing, early diagnosis and treatment, as well as prevention, are important.
Abdomen ; Anemia ; Chills ; Drug Therapy ; Early Diagnosis ; Female ; Fever ; Humans ; Korea ; Malaria ; Malaria, Vivax* ; Male ; Medical Records ; Platelet Count ; Retrospective Studies ; Splenic Infarction ; Splenomegaly ; Thrombocytopenia ; Ultrasonography

PURPOSE: Splenomegaly is a clinical surrogate of oxaliplatin-induced sinusoidal obstruction syndrome (SOS). We investigated development of splenomegaly and its association with treatment outcome and genetic polymorphisms following adjuvant 5-fluorouracil, leucovorin, and oxaliplatin (FOLFOX) in colorectal cancer (CRC) patients. MATERIALS AND METHODS: Splenomegaly was determined by spleen volumetry using computed tomography images obtained before initiation of chemotherapy and after completion of adjuvant FOLFOX in CRC patients. Ten genetic polymorphisms in 4 SOS-related genes (VEGFA, MMP9, NOS3, and GSTP1) were analyzed using DNA from peripheral blood mononuclear cells. RESULTS: Of 124 patients included, increase in spleen size was observed in 109 (87.9%). Median change was 31% (range, -42% to 168%). Patients with splenomegaly had more severe thrombocytopenia compared to patients without splenomegaly during the chemotherapy period (p < 0.0001). The cumulative dose of oxaliplatin and the lowest platelet count during the chemotherapy period were clinical factors associated with splenomegaly. However, no significant associations were found between genetic polymorphisms and development of splenomegaly. Disease-free survival was similar regardless of the development of splenomegaly. CONCLUSION: Splenomegaly was frequently observed in patients receiving adjuvant FOLFOX and resulted in more severe thrombocytopenia but did not influence treatment outcome. Examined genetic polymorphisms did not predict development of splenomegaly.
Colorectal Neoplasms* ; Disease-Free Survival ; DNA ; Drug Therapy ; Fluorouracil ; Hepatic Veno-Occlusive Disease ; Humans ; Leucovorin ; Platelet Count ; Polymorphism, Genetic* ; Spleen ; Splenomegaly* ; Thrombocytopenia ; Treatment Outcome*