Sheehan's syndrome associated with Glanzmann's Thrombasthenia: case report and literature review.

OBJECTIVES: We aimed to present a case of Sheehan's syndrome associated with Glanzmann's thrombasthenia. CASE REPORT: A 41-year-old-woman presented with hemorrhagic diathesis needed blood transfusions occasionally during her life-time. In the course of her second delivery, four units of whole blood transfusion were required to control profuse uterine bleeding and her hemodynamic status. During postpartum period, she was not able to lactate and her menstrual periods did not return. Her hormonal profile revealed gonadotropin, TSH and prolactin deficiencies. CONCLUSION: According to our knowledge, this is the first case report of hypopituitarism developed after delivery in association with Glanzmann's thromboasthenia-induced severe uterine hemorrhage.

Time-related changes in the incidence, severity, and clinical outcome of hepatic veno-occlusive disease in hematopoietic stem cell transplantation patients during the past 10 years.

Veno-occlusive disease (VOD) of the liver occurs in 10% to 50% of patients after hematopoietic stem cell transplantation (HSCT), ranging from a mild reversible disease to a fulminant course with a mortality rate close to 100%. We retrospectively evaluated the clinical signs, diagnosis, prognosis, therapy, and outcome of 13 hepatic VOD cases which developed after HSCT. A total of 193 consecutive patients (age: 15-62 years; median 33 years) with various hematologic diseases underwent 197 HSCT (allogeneic HSCT, n = 128; autologous HSCT, n = 69). In general, the conditioning regimen consisted of cyclophosphamide combined either with total body irradiation or busulfan. Since 2000, to reduce hepatic complications, all patients received ursodexycolic acid and discontinuation of norethisterone which inhibits ovulation. VOD diagnosed clinically was mainly managed in supportive fashion. Five patients received thrombolytic therapy (t-plasminogen activator [t-PA], n = 3; defibrotide [DF], n = 2). VOD developed in 13 of 197 cases (6.6%). All except one were in the allogeneic group who had received a busulfan-containing conditioning regimen; Ten (77%) were severe. Thirty-three of 197 (17%) cases died before day 100 with VOD as the cause in eight (24%). All of the t-PA administered patients died with significant hemorrhagic complications. DF patients improved completely, even after renal and respiratory failure, despite high total bilirubin levels. Only one patient who received DF became a long-term survivor; the other died with sepsis during the following days. The dramatic improvement with regard to VOD during DF therapy was encouraging.

Peritoneal seeding and subsequent progression of mantle cell lymphoma after splenectomy for debulking.

BACKGROUND: Peritoneal seeding after abdominal surgery is a well known route of metastasis in intra-abdominal solid tumours. Direct mechanical contamination, local peritoneal trauma and subsequent inflammation, postoperative immunosuppression, and laparoscopic surgery are the proposed predisposing factors for this type of metastasis. These factors probably result in enhanced adhesion or growth of tumour cells. However, this route of metastasis has not yet been reported for lymphomas. Here, we report the first case of peritoneal seeding of lymphoma cells after an abdominal surgery. CASE DESCRIPTION: A 47-year-old man with mantle cell lymphoma had ascites because of infiltration of the liver. He underwent debulking splenectomy. The postoperative ascites cytology and control abdominal computed tomography imaging both confirmed peritoneal involvement and lymphoma progression. Demonstration of negative peritoneal involvement before surgery and close timing of peritoneal involvement after splenectomy suggested to us that the debulking surgery was the main cause of peritoneal seeding of lymphoma cells in our case. CONCLUSIONS: Factors similar to those in solid tumour seeding may also be valid for lymphomas. Peritoneal seeding and consequent disease progression may be a potential complication of abdominal surgery in lymphoma with extensive intra-abdominal involvement.

Myeloid antigen expression provides favorable outcome in patients with adult acute lymphoblastic leukemia: a single-center study.

Between July 1992 and July 2001, 81 patients with de novo adult acute lymphoblastic leukemia (ALL) treated according to the German Multicenter Study Group for Adult ALL (GMALL) 01/81 protocol were evaluated in order to analyze the effect of aberrant myeloid antigen expression on prognosis. We observed myeloid antigen aberrant expression in 21 of the adult ALL cases. We did not observe any effect of aberrant myeloid antigen expression on the time to achieve remission, relapse rate, and death rate. After 5 years of follow-up, cumulative disease-free survival of myeloid antigen (My) (+) and My (-) adult ALL patients was 67% and 43%, respectively. These data were not found to be statistically significant (P=0.29), but we did find a statistically significant difference in overall survivals between these two groups (85% vs 50%) (P=0.05). Twenty-nine patients died and the remaining 52 patients were followed for a median of 31 months. We could not find any special effect of the known prognostic factors on prediction of relapse in multivariate analysis. However, myeloid antigen expression was the most significant factor, which affected long-term survival in our patients (P=0.01). These data indicate that myeloid antigen expression is useful for predicting a favorable outcome of adult patients with ALL.