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Biomedical subjects

A Manoharan

Publications and source records attributed to A Manoharan.

At least 145 records · Page 8Linked to original sources

Non-tropical idiopathic splenomegaly (Dacie's syndrome): report of 5 cases.

We describe 5 patients, aged 22-69 years, with massive splenomegaly of unknown origin an features of hypersplenism. Splenectomy corrected the cytopenia(s) and abolished the symptoms in each case; the histological features of the spleen were non-specific and included congestive changes and lymphoid hyperplasia. 2 of these patients developed non-Hodgkin's lymphoma (NHL) 2 and 6 years after spenectomy. A total of 46 cases of splenomegaly of unknown origin have been reported from UK, USA, and Australia so far, and in 9, NHL developed 8 to 80 months after splenectomy. The questions relating to the pathogenesis of splenomegaly and the subsequent development of NHL remain to be answered. We propose that this 'entity' be known as Dacie's syndrome, after Sir John Dacie who characterised it in 1969.

Adult↗

Prognostic factors in myelofibrosis.

The prognostic significance of spleen size, hemoglobin level (Hb), platelet count and hemopoietic cellularity of the bone marrow was assessed in 33 patients with myelofibrosis. Of these 4 parameters, only the Hb (P less than 0.01) and bone marrow cellularity (P less than 0.001) were found to be useful indicators of prognosis. Patients with Hb greater than 10 g/dl had a median survival greater than 110 mth while those with Hb less than 10 g/dl had a median survival of 22.5 mth; patients with hemopoietic hypercellularity in the bone marrow had a median survival greater than 110 mth while those with a normo- or hypocellularity had a median survival of 20.5 mth. There was significant correlation (P less than 0.01) between the marrow cellularity and the Hb level; 71% of patients with a normo- or hypocellular marrow had Hb less than 10 g/dl, while 79% of those with a hypercellular marrow had Hb greater than 10 g/dl.

Adult↗

Simultaneous or spontaneous occurrence of lympho- and myeloproliferative disorders: a report of four cases.

We describe four patients with mixed lympho- and myeloproliferative disorders. One patient had hairy cell leukaemia and acute myelomonocytic leukaemia, another lymphocytic lymphoma in leukaemic phase and chronic myelomonocytic leukaemia and the third patient had chronic lymphocytic leukaemia and polycythaemia rubra vera; none of these patients had received any prior therapy, and in two the diagnosis of the two malignancies was simultaneous. The fourth patient developed acute monocytic leukaemia 4 years after the diagnosis of chronic lymphocytic leukaemia after only 2 weeks of therapy with chlorambucil. The nuber of cases with concurrent or sequential but spontaneous occurrence of lympho- and myeloproliferative disorders reported so far is now 38. The questions relating to the pathogenesis of the two malignancies are discussed.

Aged↗

Histiocytic medullary reticulosis complicating chronic lymphocytic leukaemia: malignant or reactive?

We describe 4 patients with a haemophagocytic syndrome resembling histiocytic medullary reticulosis (HMR) complicating chronic lymphocytic leukaemia (CLL) of 9 months to 8 years duration. Surface marker studies in 2 cases showed that the CLL lymphocytes were of B-cell type in one and of T-cell type in the other. 2 of the patients had histiological evidence of co-existing immunoblastic sarcoma at the time of diagnosis of the HMR-like syndrome and all 4 patients died within 3 weeks of this diagnosis. The pathogenesis of the HMR-like syndrome in these patients is discussed and it is concluded that it is probably reactive to an underlying opportunistic viral function related to their immunodepressed state secondary to CLL and/or the cytotoxic therapy.

Adolescent↗

Myelomatosis in aplastic anaemia--a true association or fortuitous occurrence?

A patient with aplastic anaemia subsequently developed multiple myeloma. This unusual association may have been coincidental but a possible link is discussed. Should similar cases occur, it is important that they be documented, as more experimental and clinical evidence is necessary before lymphoid malignancies are related to bone marrow injury.

Aged↗

Significance of splenomegaly in childhood acute lymphoblastic leukaemia in remission.

In 5 children with acute lymphoblastic leukaemia (ALL) splenomegaly occurred or persisted after induction of haematological remission. 3 patients underwent splenectomy but the spleen was free of leukaemia in each case; these patients relapsed and died 10 to 28 months after surgery. 2 patients who were not splenectomised are alive and free of leukaemia 2 and 6 years after splenomegaly was first noted; in 1 splenomegaly regressed after cessation of antileukaemic therapy. The isolated finding of splenomegaly in children with ALL in haematological remission does not necessarily indicate a relapse; instead, it is suggested that splenic enlargement may reflect an immunological process contributing to control the leukaemia and that removal of the spleen may be harmful.

Child↗

Radiation pneumonia complicating adjuvant cytotoxic chemotherapy.

A patient with Hodgkin's disease developed acute radiation pneumonia four months after completion of mantle radiotherapy and during cyclical adjuvant chemotherapy. Respiratory function tests were useful in the diagnosis of the interstitial abnormality and in monitoring response to prednisone. Serial respiratory function tests in patients receiving bimodal therapy may facilitate early diagnosis of this potentially serious complication.

Adult↗

The reticulin content of bone marrow in acute leukaemia in adults.

Marrow reticulin was studied by trephine biopsy in 44 patients with adult acute leukaemia at presentation and subsequently during the course of their illness. The findings indicate that: (1) an increase in marrow reticulin is common at presentation in patients with both acute lymphoblastic and acute non-lymphoblastic leukaemia; (2) effective anti-leukaemic therapy results in resolution of some or all of the increased marrow reticulin and is not contraindicated, even in patients with a marked increase in marrow reticulin; and (3) reappearance of an increase in marrow reticulin may be a sign of relapse of the leukaemia.

Acute Disease↗

Adenocarcinoma of the lung in non-Hodgkin's lymphoma.

The development of a second neoplasm is a rare complication in patients with various types of primary malignancy. This report describes two patients with non-Hodgkin's lymphoma who developed adenocarcinoma of the lung and malignant pleural effusion following many years of cytotoxic therapy. The value of cytological examination of the sputum and pleural aspirate, as well as fibreoptic bronchial biopsy in the diagnosis are emphasised. The higher incidence of this complication in patients with lymphocytic type of non-Hodgkin's lymphoma may be due to their longer survival and probable basic immune defects which become overt after chemotherapy.

Adenocarcinoma↗

Acute non-lymphocytic leukaemia in the third trimester of pregnancy.

A 28-year-old woman with acute myeloblastic leukaemia was effectively treated with cytosine arabinoside and 6-thioguanine during the third trimester of pregnancy and a complete remission achieved. A normal infant was delivered at term. The patient survived for 27 months and the baby is now 2 1/2 years old with normal physical and mental development. The literature on management of acute non-lymphocytic leukaemia during the second and third trimester of pregnancy is reviewed and it is concluded that effective combination chemotherapy improves fetal survival.

Adult↗

Intrathoracic manifestations in non-Hodgkin's lymphoma.

A total of 16 of 86 patients (19%) with non-Hodgkin's lymphoma were found to have intrathoracic disease in this retrospective study. Paratracheal, mediastinal, and hilar lymphadenopathy was the commonest manifestation followed by pulmonary lesions and pleural effusion. The lymphoma was at an advanced clinical stage in all the patients with intrathoracic disease. About one-third of the intrathoracic lesions first developed at the time of relapse after successful initial therapy. There was a better response to therapy when intrathoracic disease was part of the initial presentation than when it was a manifestation of relapse. If it did not respond to therapy it was always indicative of a poor prognosis.

Adult↗

Acute myeloblastic leukaemia with marrow fibrosis (malignant myelosclerosis): acute leukaemia or malignant myelosclerosis?

Malignant myelosclerosis has been described as a rare form of acute myeloproliferative disorder characterised by minimal or absent splenomegaly, cytopenias, circulating myeloblasts and diffuse marrow fibrosis, but distinction between this entity and acute myeloblastic leukaemia with marrow fibrosis is difficult. This report describes a 50-year-old man who presented with features similar to those of malignant myelosclerosis but ther terminal stage was characterised by generalised lymphadenopathy, bone tenderness, marked leucocytosis and myeloblastaemia. Post mortem examination showed multiple chloromata consisting of myeloblasts. These findings suggest that malignant myelosclerosis may be but a variant of acute myeloblastic leukaemia and probably should be treated similarly. A possible association between diagnostic medical irradiation and this acute myeloproliferative disorder is discussed.

Acute Disease↗