Biomedical subjects

A Tefferi

Publications and source records attributed to A Tefferi.

206 records · Page 12Linked to original sources

PubMed1989

Acute megakaryocytic leukemia (M7) in children.

We analyzed the clinical and laboratory features of eight children (median age, 20 months; range, 13 months to 11 years) with acute megakaryocytic leukemia (M7) and compared the findings with those reported in the literature. The diagnosis was supported by ultrastructural examination for platelet peroxidase or immunophenotyping for glycoprotein IIb/IIIa or the von Willebrand factor protein. Two patients had Down's syndrome. Initial findings included anemia (in all patients), thrombocytopenia (in six), myelofibrosis (in three), lytic bone lesions (in two), and pronounced leukocytosis (in one). Stem cell culture studies of peripheral blood specimens revealed an aberrant phenotype of the megakaryocytes in one patient and reversal to a normal pattern after successful therapy. Remission was achieved in seven of the eight patients after aggressive chemotherapy, and four patients remained in remission 27 to 57 months after diagnosis. Three of these four patients underwent allogeneic bone marrow transplantation. M7 leukemia is not infrequent in children younger than 3 years of age, especially in those with Down's syndrome. The availability of monoclonal antibodies specific to restricted antigens of the megakaryocytic lineage has made the diagnosis of M7 leukemia both possible and practical.

Anemia↗

PubMed1989

Correlation of clinical outcome with the degree of surface immunoglobulin (sIg) expression in B-cell chronic lymphocytic leukemia.

Peripheral blood from 121 consecutive adult patients with B-cell chronic lymphocytic leukemia (B-CLL) was immunophenotyped, and three groups with differing surface immunoglobulin (sIg) expression patterns were identified: Group A, 82 patients (68%) with weak sIg expression in greater than 20% of the circulating lymphocytes; Group B, 16 patients (13%) with either undetectable sIg or weak sIg expression limited to less than 20% of the lymphocytes; and Group C, 23 patients (19%) with strong sIg expression in greater than 20% of the lymphocytes. The histories of these patients were reviewed retrospectively, and the median follow-up in Groups A, B, and C was nine, ten, and nine years, respectively. Disease progression, defined as the development of cytopenia(s) (hemoglobin less than or equal to 100 g/L [less than or equal to 10 g/dL] or platelet count less than 100 X 10(9)/L [less than 100 X 10(3) microL]) and/or the development of newly palpable splenomegaly or lymphadenopathy, was least in Group B (6%) versus Group A (34%) versus Group C (30%) (P = 0.08). The incidence of hypogammaglobulinemia among patients who had protein electrophoresis performed was 7% in Group B versus 46% in Group A versus 45% in Group C (P = 0.036). The authors conclude that adult patients with B-CLL in whom sIg is undetectable or weakly expressed in less than 20% of the circulating lymphocytes may have an even more indolent clinical course than those with more widespread sIg expression.

Adult↗

PubMed1989

Transient acantholytic dermatosis associated with lymphomatous angioimmunoblastic lymphadenopathy.

Transient acantholytic dermatosis is a papulovesicular cutaneous eruption first described in 1970. There have been subsequent reports of similar disorders occurring in patients with malignancy. Angioimmunoblastic lymphadenopathy with dysproteinemia is a disorder characterized by an acute onset of generalized lymphadenopathy associated with fever, malaise, pruritus, night sweats, and hepatosplenomegaly. The patient described had a papular acantholytic dermatosis associated with the development of angioimmunoblastic lymphadenopathy with dysproteinemia-like T-cell lymphoma. The cutaneous manifestations of angioimmunoblastic lymphadenopathy with dysproteinemia are discussed.

Acantholysis↗

PubMed1989

Isolation of a heparin-like anticoagulant from the plasma of a patient with metastatic bladder carcinoma.

A 73-year-old woman with metastatic transitional cell carcinoma of the bladder developed vaginal bleeding a few days after undergoing radical cystectomy. She had no other signs of mucocutaneous bleeding. Coagulation studies revealed a markedly prolonged thrombin time (greater than 600 seconds), a slightly prolonged reptilase time (20 seconds), and mildly elevated fibrinogen (4.39 g/L), and fibrin D-dimer (200 to 500 ng/mL) levels. Treatment of the patient's plasma in vitro with protamine or barium sulfate normalized the thrombin time. The anticoagulant activity corresponded to 0.15 heparin U/mL when measured by a thrombin time assay using normal plasma as substrate and standardized with porcine heparin. The anticoagulant was quantitatively bound to and subsequently eluted with 1 mol/L NaCl from quaternary aminoethyl (QAE) Sephadex, and then isolated by affinity chromatography on immobilized antithrombin III. The isolated anticoagulant was shown to be sensitive to heparinase digestion. Therefore, the inhibitor has functional and chemical properties similar to those of high-affinity heparin. Thus far, this is the only anticoagulant of this type isolated from the plasma of a patient bearing a tumor other than plasma cell myeloma.

Aged↗

PubMed1988

Role of immunotyping in chronic lymphocytosis: review of the natural history of the condition in 145 adult patients.

We investigated the clinical value of immunotyping in 145 consecutive adult patients with absolute or relative lymphocytosis: 132 (91%) had B-cell lymphocytosis, 5 (4%) had T-cell lymphocytosis, 2 (1%) had hairy cell leukemia, and 6 (4%) had reactive lymphocytosis. Of the five patients with T-cell lymphocytosis, four were best categorized as having T gamma-chronic lymphoproliferative disease and had an indolent clinical course. Of the 132 patients with B-cell lymphocytosis, 121 (92%) had B-cell chronic lymphocytic leukemia (B-CLL), and 11 (8%) had small cleaved ("lymphosarcoma") cell leukemia. Patients with small cleaved cell leukemia had a worse clinical outcome than did those with B-CLL. We further analyzed the surface immunoglobulin (sIg) and CD20 (B-1) antigen expression patterns in B-CLL to determine whether any correlation existed with clinical outcome. A subset of patients with B-CLL in whom sIg was expressed in less than 20% of their lymphocytes had the best clinical outcome. HLA-DR (Ia-like) antigen typing helped identify B-CLL cases with minimal or no sIg expression. CD20 (B-1) antigen was weak or undetectable in most cases of B-CLL. Patients with B-CLL who had CD20 (B-1) in more than 20% of their lymphocytes did not have a different prognosis. Our data provide the incidence and natural history of the various subsets of CLL in a series of patients at a single institution. The type and extent of immunotyping necessary and practical in the clinical management of patients with CLL are explored.

Adult↗

PubMedDate not supplied

Palliative splenectomy in myelofibrosis with myeloid metaplasia.

Myelofibrosis with myeloid metaplasia (MMM) is a chronic myeloproliferative disorder in which the accumulation and growth of circulating myeloid progenitors in the spleen lead to pathologic enlargement of the organ with resulting mechanical discomfort, hypercatabolic symptoms, anemia, thrombocytopenia, and portal hypertension. Medical therapy and splenic irradiation may be of benefit in certain patients, yet many may still require splenectomy to palliate their symptoms. Although there is no clear survival advantage to splenectomy in MMM, the procedure can result in substantial palliation of symptoms. However, the surgical procedure is associated with an approximately 9% mortality rate, and the postsplenectomy occurrence of extreme thrombocytosis, hepatomegaly, and leukemic transformation is of major concern. The management of splenomegaly and the role of splenectomy in MMM are discussed in this review.

Disease Management↗

PubMedDate not supplied

Myelodysplastic syndrome and pregnancy: the Mayo Clinic experience.

Although the myelodysplastic syndrome (MDS) is most common in the elderly, younger patients, including women of child-bearing age, may be affected. The association of MDS with pregnancy appears to be very rare: fewer than 25 cases have been reported. We report the outcomes of seven pregnancies in four women seen at the Mayo clinic between 1983 and 2000. Three of the women were found to have MDS when an abnormal complete blood count was detected during routine prenatal care. The fourth patient had an apparently congenital MDS, and suffered three spontaneous abortions before undergoing premature menopause as a result of pelvic irradiation for vulvar cancer. We discuss some of the unique concerns regarding pregnant patients with MDS. As women give birth at older ages and as more young persons survive cancer, MDS and pregnancy are likely to be seen together with increasing frequency.

Adult↗

PubMedDate not supplied

Hydroxyurea-associated platelet count oscillations in polycythemia vera: a report of four new cases and a review.

Cyclic oscillations in the peripheral blood platelet count were recently described in two patients with polycythemia vera (PV) receiving therapy with hydroxyurea (HU). This phenomenon can make proper HU dosing very challenging and may be especially problematic in PV patients who are at risk for thrombohemorrhagic complications. In this report, we describe four new cases of HU-associated platelet oscillations in patients with PV and extend our observations on one of the two previously reported cases. We also review cyclic thrombocytosis within the broader context of periodic hematopoiesis. A thrombopoietin-mediated negative feedback loop is central to most models of periodic thrombopoiesis, but this is probably an oversimplification. It is possible that HU destabilizes the delicate balance of thrombopoietin/c-Mpl signaling in megakaryocytic lineage hematopoiesis that is already markedly altered in PV; this hypothesis remains speculative. For patients who develop oscillatory variation in their platelet counts associated with HU use, keeping the HU dose constant may result in damping or termination of the cycles. However, this strategy is not always successful.

Adult↗