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Biomedical subjects

Shaji Kumar

Publications and source records attributed to Shaji Kumar.

42 records · Page 3Linked to original sources

Acute superior mesenteric venous thrombosis: one disease or two?

OBJECTIVE: The aim of this study was to determine the etiology and natural history of acute superior mesenteric venous thrombosis (MVT) with and without splenic or portal vein involvement. METHODS: A retrospective analysis was carried out of patients with acute superior MVT evaluated between 1979 and 1998. Case records were reviewed and a questionnaire mailed to patients for follow-up evaluation. Also, a search was made to identify etiologies such as malignancy, infection, inflammatory bowel disease, and other risk factors. Patients were divided into two groups, isolated MVT or MVT with splenic or portal vein involvement. RESULTS: A specific etiology (malignancy, thrombophilia, inflammatory bowel disease, or surgery) was found in 35 of 69 patients. Thirty patients had isolated MVT with involvement predominantly of the small mesenteric veins; in the remaining 39 there was also portal or splenic vein involvement. Patients with isolated MVT were less likely to be diagnosed by imaging studies such as ultrasonography and CT and more likely to have bowel necrosis, and they required surgery more frequently. Inherited hypercoagulable disorders were more often found in patients with isolated MVT. During follow-up, six patients had recurrent MVT, and one patient with combined mesenteric and portal vein thrombosis developed variceal bleeding. CONCLUSIONS: Patients with isolated MVT are more likely to have hypercoagulable disorders. Isolated MVT is more difficult to diagnose and more likely to require surgery. Classification of patients with MVT into those with small vessel involvement or those with splenic or portal vein involvement thus has important clinical and prognostic value. Patients surviving the initial period have a good prognosis but definite risk of recurrence. Risk of variceal complications appears low.

Acute Disease↗

Response rate, durability of response, and survival after thalidomide therapy for relapsed multiple myeloma.

OBJECTIVE: To assess response rate, duration of response, progression-free survival, and toxicity of thalidomide in patients with relapsed multiple myeloma. PATIENTS AND METHODS: Thirty-two patients with relapsed multiple myeloma were entered into the study between April 29, 1999, and June 20, 2000. They were given oral thalidomide at a dosage of 200 mg/d for 2 weeks, which was then increased as tolerated to a maximum of 800 mg/d. The primary end point of the study was response rate. RESULTS: The median age of the patients was 67 years (range, 36-78 years). Prior chemotherapy had failed in all patients, and stem cell transplantation had failed in 5 patients (16%). There were 10 confirmed responses, yielding a response rate of 31%. In addition, there was 1 unconfirmed partial response and 7 minimal responses with no complete responses. The median duration of response was 11.9 months (range, 3.7-203 months). Overall, 20 patients have died, and 26 patients have experienced disease progression. The median follow-up of surviving patients was 28.5 months (range, 193-34.0 months), with a median progression-free survival of 8.6 months (95% confidence interval [CI], 4.7-16 months). The median progression-free survival among the responding patients was 15.7 months (95% CI, 8.6-25.6 months). The median overall survival for the entire group was 22 months (95% CI, 10.6-35.9 months). The most common treatment-related nonhematologic toxic effects (grade >3) were neuropathy (16%), sedation (13%), febrile neutropenia (6%), and constipation (6%). CONCLUSIONS: Thalidomide is useful in the treatment of patients with relapsed multiple myeloma and produced durable response in approximately one third of patients, with median response duration of nearly 1 year.

Adult↗

Hepatic veno-occlusive disease (sinusoidal obstruction syndrome) after hematopoietic stem cell transplantation.

Hepatic veno-occlusive disease (VOD), increasingly referred to as sinusoidal obstruction syndrome, is a well-recognized complication of hematopoietic stem cell transplantation and contributes to considerable morbidity and mortality. In the Western Hemisphere, VOD, classified as a conditioning-related toxicity, is most commonly caused by stem cell transplantation. VOD has been described after all types of stem cell transplantation, irrespective of the stem cell source, type of conditioning therapy, or underlying disease. Recognition of this disease in the posttransplantation setting remains a challenge in the absence of specific diagnostic features because many other more common conditions can mimic it. Limited therapeutic or preventive strategies are currently available for the management of VOD. In this review, we provide a comprehensive account of the pathophysiology of this disease as we understand it today, risk factors for its development, and the current state of knowledge regarding preventive and therapeutic options.

Diagnosis, Differential↗

Prognostic value of angiogenesis in solitary bone plasmacytoma.

Angiogenesis plays an important role in the biology of multiple myeloma (MM) and has prognostic importance in this disease. Solitary plasmacytoma is a localized plasma cell malignancy that progresses to MM in a significant number of patients. We examined if angiogenesis is increased in solitary plasmacytoma and if it can help identify patients likely to progress to myeloma. We studied angiogenesis in plasmacytoma biopsy samples and bone marrow biopsies from 25 patients. High-grade angiogenesis was present in 64% of plasmacytomas. In contrast, bone marrow angiogenesis was low in all patients. Patients with high-grade angiogenesis in the plasmacytoma sample were more likely to progress to myeloma and had a shorter progression-free survival compared with patients with low-grade angiogenesis (P =.02). Angiogenesis is increased in solitary plasmacytoma and is a significant predictor of progression to myeloma and provides further evidence of its importance in the pathogenesis of myeloma.

Adult↗

Bone marrow angiogenesis in multiple myeloma: effect of therapy.

Recent studies have demonstrated that angiogenesis has a role in haematological malignancies, including multiple myeloma. Multiple myeloma is characterized by inevitable relapse after standard or high-dose chemotherapy. To study the effect of chemotherapy on bone marrow angiogenesis in patients with multiple myeloma, we used two methods to evaluate bone marrow angiogenesis in patients with newly diagnosed multiple myeloma, comparing these findings with those from bone marrow obtained after standard chemotherapy. Before therapy, an increased degree of bone marrow angiogenesis and a high bone marrow plasma cell labelling index (PCLI) were predictive of poorer survival. As estimated by microvessel density (MVD), the median survivals for patients with low-grade, intermediate-grade and high-grade angiogenesis were 77, 30 and 14 months respectively. After therapy, the MVD did not change significantly. However, when patients with at least a partial response were considered separately, they showed a decrease in MVD. Post-therapy PCLI was predictive of survival, but post-therapy MVD was not. There was good correlation between angiogenesis estimated by visual grading and that determined by MVD assessment. We conclude that the degree of bone marrow angiogenesis is a prognostic marker in patients with multiple myeloma and does not decrease significantly after therapy.

Adult↗

Acquired von Willebrand disease.

Acquired von Willebrand disease (AvWD) is a relatively rare acquired bleeding disorder that usually occurs in elderly patients, in whom its recognition may be delayed. Patients usually present predominantly with mucocutaneous bleeding, with no previous history of bleeding abnormalities and no clinically meaningful family history. Various underlying diseases have been associated with AvWD, most commonly hematoproliferative disorders, including monoclonal gammopathies, lymphoproliferative disorders, and myeloproliferative disorders. The pathogenesis of AvWD remains incompletely understood but includes autoantibodies directed against the von Willebrand factor (vWF), leading to a more rapid clearance from the circulation or interference with its function, adsorption of vWF by tumor cells, and nonimmunologic mechanisms of destruction. Laboratory evaluation usually reveals a pattern of prolonged bleeding time and decreased levels of vWF antigen, ristocetin cofactor activity, and factor VIII coagulant activity consistent with a diagnosis of vWD. Acquired vWD is distinguished from the congenital form by age at presentation, absence of a personal and family history of bleeding disorders, and, often, presence of a hematoproliferative or autoimmune disorder. The severity of the bleeding varies considerably among patients. Therapeutic options include desmopressin and certain factor VIII concentrates that also contain vWF. Successful treatment of the associated illness can reverse the clinical and laboratory manifestations. Intravenous immunoglobulins have also shown some efficacy in the management of AvWD, especially cases associated with monoclonal gammopathies. Awareness of AvWD is essential for diagnosis and appropriate management.

Age Distribution↗