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Biomedical subjects

G J Mufti

Publications and source records attributed to G J Mufti.

At least 145 records · Page 8Linked to original sources
PubMed1986

Myelodysplastic syndromes: their history, evolution and relation to acute myeloid leukaemia.

The myelodysplastic syndromes (MDS) constitute a heterogeneous group of clonal disorders arising from a multipotent haemopoietic progenitor which share a leukaemic propensity, 30% of cases culminating in acute myeloid leukaemia (AML). Their pathogenesis probably entails multiple steps, phenotypic progression being determined by either expansion or evolution of the abnormal clone. The clonal origin of certain cases of de novo AML is analogous to that of MDS and evidence that they share a common pathogenesis and distinct biological characteristics is beginning to emerge.

Chromosome Aberrations↗
PubMed1986

Immunological abnormalities in myelodysplastic syndromes. I. Serum immunoglobulins and autoantibodies.

The immunoglobulin levels and autoantibody profiles of 104 patients with primary myelodysplastic syndromes (MDS), classified according to the FAB criteria, were analysed. Eight patients, four with coexistent non-Hodgkin's lymphomas, three with chronic lymphocytic leukaemia and one with a lymphoplasmacytoma, were excluded from the final analysis of immunoglobulin levels. Serum protein electrophoresis and immunoelectrophoresis revealed the presence of monoclonal gammopathy in 12 patients (12.5%). Of the remaining 84 patients, a polyclonal rise in serum immunoglobulins was present in 27 (32%) while a further 16 (19%) had low immunoglobulin levels. The direct antiglobulin test was positive in eight out of 98 patients tested (8.1%), and organ and non-organ specific autoantibodies were present in 15 out of 67 patients tested (22.3%). Two patients had associated pernicious anaemia (PA), two hypothyroidism, and one PA with hypothyroidism. Three patients had sero-negative rheumatoid arthritis. These results demonstrate that there is a high incidence of immunological abnormalities in MDS.

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PubMed1986

Immunological abnormalities in myelodysplastic syndromes. II. Coexistent lymphoid or plasma cell neoplasms: a report of 20 cases unrelated to chemotherapy.

We report a series of 20 patients with the myelodysplastic syndrome (MDS) each with a coexistent lymphoid or plasmacytic neoplasm. In none of the patients did chemotherapy play a part in the pathogenesis. The possible reasons for the coincidence of these conditions are discussed. The frequency and variety of associated neoplasms reinforces the idea that in MDS there are genetically unstable progenitor cells liable to clonal differentiation along a variety of pathways given appropriate stimuli.

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PubMed1986

Monocytic skin infiltration in chronic myelomonocytic leukaemia.

Although leukaemic infiltration of the skin commonly occurs in acute monoblastic leukaemia, the association with chronic myelomonocytic leukaemia (CMML) has only been described recently. We confirm the association and report histologically proven monocytic infiltration of the skin in four patients with CMML. This did not herald a more aggressive phase of the disease as previously suggested. Treatment of the rash with low dose cytarabine or etoposide was effective but razoxane produced no benefit. Superficial radiotherapy was useful to control pruritus in one patient.

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PubMed1986

Evolution of a terminal deoxynucleotidyl transferase-positive lymphoma from a chronic T cell lymphocytosis.

A 56-yr-old Caucasian man presented with a generalised scaly rash and a peripheral blood lymphocytosis of 5.6 X 10(9)/l. 5 yr later he developed cutaneous nodules, lymphadenopathy and hepatosplenomegaly. Cells with convoluted nuclei and prominent nucleoli were seen in the peripheral blood. He underwent splenectomy and received intensive chemotherapy but died 6 months later with CNS infiltration. At presentation the peripheral blood lymphocytes were E-ve, UCHT1 + ve, and OKT8 + ve. Following transformation, cells in blood, spleen and CSF were E-ve, OKT11 + ve, DR + ve and Tdt + ve. A proportion of these cells had a Sézary-like appearance at E/M. The splenic cells showed functional suppressor activity. This is the first reported case of the evolution of a Tdt + ve lymphoma from a post-thymic T cell lymphocytosis.

DNA Nucleotidylexotransferase↗
PubMed1985

Hairy cell leukaemia and Legionnaires' disease.

A 69-year-old man who had recently returned from a holiday in Majorca, presented with haemoptysis and fever. Chest radiograph showed right lower lobe consolidation. A diagnosis of Legionnaires' disease was made. He was treated with erythromycin and made an uneventful recovery.

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PubMed1985

Myelodysplastic syndromes: a scoring system with prognostic significance.

141 patients with MDS were classified according to the FAB criteria and followed up for a period of 4-192 months. It was recognized that patients with RAEBT had a uniformly poor prognosis. However, there was a wide variation in survival among the other subgroups. A score of 1 was assigned to each of the following presenting haematological features: bone marrow blasts greater than or equal to 5%, platelets less than or equal to 100 X 10(9)/l, neutrophils less than or equal to 2.5 X 10(9)/l and Hb less than or equal to 10.0 g/dl. Therefore the score for each patient ranged between 0 and 4. There were no statistically significant differences between those patients who scored 0 or 1, or between those who scored 2 and 3. Therefore patients were put into three groups: Group A (score 0 or 1), Group B (score 2 or 3), Group C (score 4). The differences in survival between each of the three groups are highly significant (P less than 0.00001). This system further separates patients with RA, RAS, RAEB into good and bad prognostic groups. This study also confirms that deaths due to cytopenias are more common than those due to transformation to AML. The use of this scoring system in conjunction with the FAB criteria for MDS should serve as a prognostic tool on which to base treatment.

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PubMed1985

Successful treatment of post-transfusion purpura with high dose immunoglobulins after lack of response to plasma exchange.

A 77-year-old woman with post-transfusion purpura failed to respond to two 2.5-litre plasma exchanges with albumin as a replacement fluid. However, intravenous infusion of high-dose human immunoglobulin produced a response within 4 h. It is suggested that plasma exchange and exchange transfusion are effective in this condition mainly because they have allowed large doses of immunoglobulin to be infused in the form of plasma or whole blood.

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PubMed1985

Reciprocal translocation between chromosomes 8 and 9 in atypical chronic myeloid leukaemia.

A balanced translocation t(8;9) (p11;q34) was present in the peripheral blood, bone marrow, and spleen cells of a patient with Ph negative chronic myeloid leukaemia. Subsequent transformation into acute leukaemia was associated with the emergence of trisomy 8 and der(8)(8qter----cen----8p11::9q34----9qter). This is the third reported case of t(8;9) (p11;q34) and raises the question of the role of c-abl in the pathogenesis of this myeloproliferative disorder.

Adult↗
PubMed1985

Angio-immunoblastic lymphadenopathy with hypogammaglobulinaemia complicated by miliary tuberculosis.

Angio-immunoblastic lymphadenopathy is a systemic disease of unknown aetiology. It carries a high mortality mainly from infection which results both from the intrinsic immunodeficiency and its exacerbation by treatment with steroids and or cytotoxics. We report a case of angio-immunoblastic lymphadenopathy with the unusual feature of hypogammaglobulinaemia who died of miliary tuberculosis. We suggest that all patients with angio-immunoblastic lymphadenopathy who have inactive tuberculous foci should be given antituberculous prophylaxis.

Agammaglobulinemia↗
PubMed1984

Serous effusions in monocytic leukaemias.

We report four patients who either presented with or developed serous effusions during the course of monocytic leukaemia. This finding has not been documented previously. All the patients had very high peripheral blood monocyte counts when effusions developed. The effusions resolved after the patients were treated with anti-leukaemic therapy. It is therefore likely that the effusions were disease related. Two of the patients have been treated with razoxane, one with etoposide and the other with cytarabine.

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PubMed1984

Relapse of lymphoma in the heart after a 10-year remission.

A 71-year-old woman presented with intractable cardiac failure 10 years after receiving treatment for lymphoma. Extensive investigations failed to demonstrate a recurrence of her disease, but resolution of her cardiac failure following a trial of chemotherapy and subsequently a cross-sectional echocardiogram suggested intracardiac relapse. This was later confirmed at autopsy. Cardiac metastases should be suspected in patients with malignant disease who develop signs or symptoms related to the heart.

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PubMed1984

Angiodysplasia of the colon in patients with hemostatic defects: risk of secondary hemorrhage after electrocoagulation treatment.

Angiodysplasia of the colon is a relatively common cause of blood loss in the elderly. Electrocoagulation through a colonoscope is a common mode of treatment for this condition. Three patients are presented who had associated hemostatic defects and electrocoagulation resulted in life threatening secondary hemorrhage. In two of these patients a right hemicolectomy was performed. On the basis of these findings we suggest that patients with angiodysplasia and associated hemostatic disorders have a greatly increased risk of secondary hemorrhage following electrocoagulation treatment. These patients should be followed closely for two-three weeks following this treatment.

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