Two Cases of Crohn's Disease with Myelodysplastic Syndrome / 대한혈액학회지

Myelodysplastic syndromes (MDS) are bone marrow stem cell disorders characterized by dysplastic hematopoiesis leading to peripheral pancytopenias, and by a high risk of progression to acute myeloid leukemia. Several immunological disorders, particularly relapsing polychondritis, seronegative arthritis and cutaneous vasculitis have been described in association with MDS. Crohn's disease is an inflammatory bowel disease characterized by inflammatory, ulcerative bowel lesions and frequent association with systemic manifestations. Recently, some researchers have suggested that an association may exist between MDS and inflammatory bowel diseases, especially Crohn's disease, based on concomittant findings of both disorders in some reported patients. We report here two cases who developed MDS and Crohn's disease concurrently, and review previously reported literatures.

Transplantation of peripheral blood stem cells mobilized by intensified consolidation and granulocyte colony-stimulating factor in acute leukemia

The purpose of this study was to evaluate the feasibility and efficacy of autologous transplantation of peripheral blood stem cells (PBSC) mobilized with high-dose consolidation chemotherapy and granulocyte colony-stimulating factor in patients with acute myelogenous leukemia (AML). Twenty patients received myeloablative chemotherapy or chemo-radiotherapy including total body irradiation followed by the infusion of PBSC. PBSC were collected by large-volume leukaphereses. The mean number of mononuclear cells and CD34-positive cells infused were 7.2 x 10(8)/kg (range, 2.2-16.6), and 6.6 x 106/kg (range, 2.1-27.7), respectively. Engraftment failure was not seen in the enrolled patients. The median time to neutrophil (> or = 500/microL) and platelet recovery (> or = 50,000/microL) from the transplant was 12 days (range, 8-20) and 28 days (range, 10-600), respectively. The 2-year probability of disease-free survival (DFS) and relapse were 43% and 57% for patients with AML transplanted in first complete remission (CR1). The outcome of the patients transplanted in the advanced status was significantly worse than the patients transplanted in CR1 (P=0.04). Most relapses occurred within 1 year after transplantation. Fatal hepatic veno-occlusive disease was observed in one case. Other transplantation-related toxicities were mild. Our results demonstrated that autologous transplantation of high-dose consolidation chemotherapy-mobilized peripheral blood progenitor cells is feasible in the patients with AML in CR1. To further reduce the risk of leukemia relapse, much effort should be contributed to the field of ex vivo purging and post-transplant immunotherapy.

A Case of Wernicke Disease in a Patient with Acute Promyelocytic Leukemia / 대한내과학회지

Wernicke's disease is a condition produced by thiamine deficiency; more than 90% of the cases are observed in chronic alcoholics. Other less frequent conditions associated with Wernicke disease are gastric disorders, prolonged parenteral nutrition, uremia, hemodialysis, hyperemesis gravidarum, prolonged starvation, and AIDS. We report a 41-year-old female patient of Wernicke disease associated with nausea and prolonged parenteral nutrition after chemotherapy of acute promyelocytic leukemia. She has got thiamine replacement therapy and most symptoms were improved. She was discharged after complete remission of leukemia and recovery of normal diet.

Vitamin B12 Deficiency Anemia after Total Gastrectomy in Patients with Stomach Cancer / 대한내과학회지

OBJECTIVES: We attempt to find out the decremental character of serum vitamin B12 level and features of patients with vitamin B12 deficiency anemia occurring earlier than 4 years after total gastrectomy. Methods: We studied 91 patients who underwent total gastrectomy and select 59 patients who evaluate serum vitamin B12 level within 4 years after total gastrectomy to characterize the features of vitamin B12 deficiency anemia group occurring earlier than 4 years after the operation. RESULTS: 1) In 91 patients, the mean postoperative duration is 31.9(range: 0-135) months and mean serum vitamin B12 level 158.9(total 159 times, range: 20.0-806.2) pg/mL. Correlation equation is y=403.30 x e-0.09x+175.93 x e-0.02 x (r2=0.804). The postoperation duration that serum vitamin B12 level falls below 200 pg/mL is 20 months. 2) Among 59 patients, there were 20 cases of vitamin B12 deficiency anemia group(Group I), 21 cases of vitamin B12 deficiency(Group II) and 39 cases of non-vitamin B12 deficiency group(Group III). Preoperation symptom dura tion in Group I was longer than that in Group II(18.2 vs 9.6 weeks, p<0.01). Group I and Group II showed lower serum vitamin B12 level than Group III(126.7 & 96.0 pg/mL vs 330.4 pg/mL, p<0.01). 3) Eleven cases had associated diseases(9 alcoholics and 2 liver cirrhosis) with vitamin B12 deficiency anemia and these disease had significant influence on the incidence of vitamin B12 deficiency anemia(p<0.01) within 4 years. Among the 30 cases who could measure iron profile, 10 patients had an iron deficiency anemia. There was a higher incidence of vitamin B12 deficiency anemia in iron deficiency anemia group(7/10, 70%) than that in non iron deficiency anemia group(7/20, 35%) but no sta tistical significance(p=0.12). 4) Although anti-cancer chemotherapy did not have a significant influence on the incidence of vitamin B12 defi ciency anemia, vitamin B12 level in anti-cancer chemo therapy group was higher than that in non-chemotherapy group(201.83 vs 127.66 pg/mL, p=0.01) at similar mean postoperation duration(27.8 vs 27.7 months, p=0.97). 5) The independent predictor of vitamin B12 deficiency anemia within 4 years were associated disease(p=0.002) and preoperation symptom duration(p=0.004). CONCLUSION: Red cell indices such as Hb or MCV did not have any clinical significances in predicting the development of vitamin B12 deficiency. To prevent vitamin B12 deficiency anemia and promote better quality of life in total gastrectomized patients with stomach can cer, careful monitoring of serum vitamin B12 level and re placement therapy should be necessary at least 20 months after operation, especially in patients with associated disease and longer preoperation symptom duration.

Avascular Necrosis of Bone Complicating Treatment of Malignant Lymphoma: Report of 6 cases / 대한암학회지

BACKGROUND: Several reports have documented the association of avascular necrosis (AVN) of bone and the treatment of malignant lymphoma with steroid-containing chemotherapy. It is important to recognize these conditions, as they can be mistaken for those of lymphomatous involvement.Cases: This report describes the experience at the Severance hospital over a 10-year period with 6 patients in whom AVN developed during or following treatment of malignant lymphoma. Four patients of non-Hodgkin's lymphoma and two of Hodgkin's disease were treated with steroid-containing chemotherapy. The predominant symptom is pain on motion or weight bearing. Symptoms leading to diagnosis of AVN developed between 5 and 27 months after starting prednisolone (mean 17.8 months), and the mean cumulative dose of prednisolone to the onset of AVN was 4,447 mg (range, 1,800~9,490 mg). All but one were involved in both hip joint. Diagnosis was based on the radiologic appearance, and in the majority radiographic changes consistent with AVN were present at the time of presentation of symptoms. Four patients received total hip replacement and two had conservative care. CONCLUSION: Patients with malignant lymphoma who developed pain on joint during or after the use of steroid-containing chemothearpy should be carefully investigated with MRI and radionuclear bone scan for early diagnosis and proper management.

Clinical Outcome of the HBsAg Positive Malignant Lymphoma Patients Following Anticancer Chemotherapy / 대한내과학회지

OBJECTIVES: Infection and replication of the hepatitis B virus are closely related to the host immunity. Anticancer chemotherapy decreases the immune response of the host, Especially, glucocorticoid can activate the replication of hepatitis B virus directly. It is well known that hepatitis B virus infection and hepatic complications are more common in patients with hematologic malignancies like malignant lymphoma. We studied the incidence of hepatitis B virus infection and hepatic complications following anti-cancer chemotherapy in patients with malignant lymphoma. METHODS: Among 224 cases diagnosed as malignant lymphoma from January 1989 to December 1993 at Yonsei University Medical Center, 77 cases tested for hepatitis B virus serology was studied. RESULTS: 1) Eighteen cases (23%) was HBsAg positive. 2) The results of hepatitis C virus serology in six cases were all negative. 3) Eight (57%) of 14 follow-up cases had hepatic complications, Two patients had fulminant hepatitis, two nonicteric hepatitis and four icteric hepatitis. 4) Interferon-alpha was administered in three cases among the patients with hepatic complications. Loss of HBeAg was observed in one case and loss of HBsAg in another case. CONCLUSION: Serious hepatic complications can be occurred following anticancer chemotherapy in HBsAg-positive patients with malignant lymphoma. Therefore, we recommend that patients being considered as candidates for anticancer chemotherapy should routinely undergo serologic test for Hepatitis B virus. In addition HBsAg-positive patients with anticancer chemotherapy should be regularly monitored for hepatic injury. And with the careful use of steroid and interferon, prolongation of survival might be searched for these patients.

Invasive Pulmonary Aspergillosis Involving Right Subclavian Artery and Chest Wall / 대한내과학회지

Invasive aspergillosis is an infection that occurs in immunocompromised patients. Its prevalence was increased in the last decade with progression of antineoplastic chemotherapy and immunosuppressive therapy after transplantation. Because it carries a high mortality and morbidity, early diagnosis and aggressive treatment are critical for successful management. In many patients, invasive aspergillosis remains confined to the lung although direct extension to pleural cavity or pericardium has been reported. However great vessel involvement is rare. Therefore we report a case of invasive aspergillosis involving right subclavian artery and chest wall in a patient after chemotherapy for acute lympoblastic leukemia.

A Case of Giant Adrenal Adenoma Presenting Primary Aldosteronism / 대한내분비학회지

Primary aldosteronism is a syndrome chracterized by hypokalemic alkalosis and hypertension. Small sized adrenal cortical adenomas have been the major cause of this syndrome in most of the patients. However, if the adrenal mass is larger than 6cm in diameter and with irregular consistency, malignancy is more favored. We experienced a patient who had a giant adrenal adenoma with primary aldosteronism. A 24-year-old female presented with hypertension, hypokalemia, low plasma renin, and high plasrna aldosterone levels, was found to have a 6×5.5×5 cm sized left adrenal tumor by MRI. Her clinical laboratory feature did not revealed any evidence of Cushing's syndrome or pheochromocytoma. Preoperatively adrenal carcinoma presenting pure adrenal aldosteronism was suspected due to large size and heterogenous signal character of the adrenal mass in radiologic study. At operation well encapsulated, round giant adrenal tumor weighing 65gm(4.5×4×4 cm) was removed. There was no evidence of metastasis with return of adrenal function to normal after surgery. Benign adrenal adenoma was confirmed by the gross morphology and the histologic features.