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Biomedical subjects

M M Reid

Publications and source records attributed to M M Reid.

At least 109 records · Page 6Linked to original sources

Severe haemolysis and renal failure in a patient with paroxysmal nocturnal haemoglobinuria.

Transfusion of about 60 ml of ABO incompatible plasma in 4 units of pooled platelets precipitated severe haemolysis, unmasking the emergence of paroxysmal nocturnal haemoglobinuria (PNH), in a patient with aplastic anaemia. In vitro tests showed that her red cells were lysed by both ABO compatible and incompatible plasma from normal donors. The behaviour of this case and the in vitro results suggest that it might be hazardous to relax the longstanding recommendation on transfusing patients with PNH by restricting the washing of blood components to those containing ABO incompatible plasma.

ABO Blood-Group System↗

Distinction between aleukaemic prodrome of childhood acute lymphoblastic leukaemia and aplastic anaemia.

AIMS: To document the features of the so-called aplastic presentation of childhood acute lymphoblastic leukaemia (ALL) and to determine whether this prodrome can be distinguished from aplasia. METHODS: The peripheral blood and bone marrow appearances of all cases of childhood ALL presenting in one health region of England in 13 years and eight months were reviewed. All cases presenting with cytopenia without circulating blasts and marrow aspirates with no infiltrate of blasts were studied in detail. RESULTS: Four of 305 (1.3%) children presented in this way. All four had reticulin fibrosis and increased cellularity in all or part of the marrow biopsy specimen. All were girls. Three had common and one surface membrane immunoglobulin positive ALL. Reassessment of this prodrome, by combining the features of four previously reported series of similar cases with the present one, highlighted the female preponderance (19 of 22 cases), bone marrow fibrosis (10 of 11 evaluable cases), prominent bone marrow lymphocytes (14 of 22 cases) and temporary recovery (all 12 evaluable cases). Six of 14 evaluable cases had bone marrow biopsy specimen appearances of apparently uniform hypocellularity, but only one of these did not have fibrosis. CONCLUSIONS: If, in addition to an aspirate, a bone marrow trephine biopsy is carried out the prodrome can be distinguished from aplasia in most cases. The similarity of this prodrome to aplastic anaemia is merely superficial. Clinicians and morphologists may fail to appreciate the implications of this mode of presentation if the term "aplastic" continues to be used to describe this aleukaemic prodrome of ALL.

Anemia, Aplastic↗

Alveolar rhabdomyosarcoma infiltrating bone marrow at presentation: the value to diagnosis of bone marrow trephine biopsy specimens.

AIMS: To describe the histological appearances of bone marrow infiltrated with rhabdomyosarcoma at presentation and to determine their value in establishing the diagnosis. METHODS: Patients presenting over seven years in the northern health region of England with rhabdomyosarcoma were studied. Bone marrow aspirates and trephine biopsy specimens taken at presentation were examined. RESULTS: Seven of 32 patients with rhabdomyosarcoma had bone marrow infiltration, resulting in marrow failure in all cases, at diagnosis. The diagnosis was established in these seven by the typical cytological appearances and immunophenotype of the infiltrating cells (all seven patients) and cytogenetic abnormalities (three patients). Histological examination of the bone marrow showed a pseudoalveolar pattern with fibrous septal bands, enlarged vascular channels, and lack of cohesion of the tumour cells within the subdivided aggregates in all seven. In four cases multinucleate giant cells, often with peripherally sited nuclei, were found. CONCLUSIONS: These histological features of infiltrated marrow are so characteristic that the diagnosis of alveolar rhabdomyosarcoma can be made, or at least suspected, in many cases even without recourse to technically difficult and expensive further investigations. Bone marrow biopsy should be a routine part of the investigation of patients with bone marrow failure and will be of particular value in the diagnosis of those with disseminated alveolar rhabdomyosarcoma.

Adolescent↗

Breast milk beta-glucuronidase and exaggerated jaundice in the early neonatal period.

Breast-feeding is associated with jaundice in the early neonatal period. Previous work has shown levels of the enzyme beta-glucuronidase in maternal breast milk to be related to infant serum bilirubin on postnatal day 21. Our aim was to establish if there was a correlation between the level of breast milk beta-glucuronidase and the degree of early (first week) neonatal hyperbilirubinaemia. A study of 55 mother and baby pairs showed that breast milk beta-glucuronidase levels had no relationship with the level of infant serum bilirubin between postnatal days 3 and 6. Breast milk beta-glucuronidase does not directly account for the early neonatal jaundice seen in breast-fed babies.

Bilirubin↗

Immunopathology of early graft-versus-host disease--a prospective study of skin, rectum, and peripheral blood in allogeneic and autologous bone marrow transplant recipients.

The immunopathological appearances of skin and rectum in 64 autologous and allogeneic recipients were determined before and after bone marrow transplantation. Patients who developed acute graft-versus-host disease were biopsied as soon as a clinical diagnosis was made. At the same time peripheral blood samples were collected for comparative analysis. Immunohistological and morphometric techniques were employed using a panel of monoclonal antibodies to T lymphocytes and subsets, B lymphocytes, natural killer cells, macrophages, and Langerhans cells. A reduction in the CD4/CD8 ratio after BMT was seen in skin and rectal biopsies from both autologous and allogeneic recipients with or without GVHD. The same pattern was observed in blood samples taken at the same time. Langerhans cells were reduced in the skin in all patients after BMT, probably by the conditioning regimen. Only a few cells expressing activation or natural killer cell markers were present and there were no changes observed in the macrophage population. This study has provided no evidence to implicate either CD4- or CD8-positive T lymphocytes as the initiators of the cellular damage in acute GVHD. The distribution of lymphocyte subsets in the blood was similar to that in the tissues, suggesting that the tissue changes reflect the pattern of lymphocyte repopulation after BMT and may have little bearing on the pathogenesis of GVHD.

Adolescent↗

Anti-Kell in pregnancy.

A review of data on haemolytic disease of the newborn (HDN) collected in Newcastle upon Tyne over 25 years revealed 194 pregnancies in which anti-Kell was the only antibody detected. Sixteen affected babies were born. None was hydropic, three had very severe disease but all survived. There were also three stillbirths, none of which had post-mortem appearances of HDN. The highest recorded anti-Kell titres in individual patients ranged from 1/1 to 1/2048 and bore no relation to the severity of the disease. Of the eight pregnancies in which amniotic fluid examination predicted a high risk of stillbirth, half resulted in unaffected babies. We suggest that haemolytic disease caused by anti-Kell is less severe than suggested by some workers. The use of guidelines developed from the study of Rhesus disease to determine the need for intervention in women with anti-Kell may be inappropriate.

Amniotic Fluid↗

Nutrition and bronchopulmonary dysplasia.

Twenty two babies who developed bronchopulmonary dysplasia were compared with 22 babies matched for gestational age who did not. Those with bronchopulmonary dysplasia weighed less at birth and had lower energy intakes from day 7 to day 56. Undernutrition before and after birth is a major problem in babies who develop bronchopulmonary dysplasia.

Birth Weight↗

Congenital sideroblastic anaemia in two girls.

Transfusion dependent congenital sideroblastic anaemia occurred in infancy in two unrelated girls. One girl developed early organ failure which was not prevented by standard chelation treatment. The combination of modest iron burden and putative intrinsic mitochondrial dysfunction could have accounted for the clinical picture. The other girl remained well, receiving regular transfusion and standard chelation treatment. She had normal liver function and no other evidence of organ damage. The syndrome is unlikely to be due to extreme lyonisation in carriers of the usual X-linked condition. The contrasting clinical patterns seen in these two patients suggest that transfusion dependent congenital sideroblastic anaemia may comprise a heterogeneous group of disorders. It is suggested that such children be carefully monitored for evidence of increasing iron overload so that organ damage can be prevented.

Anemia, Sideroblastic↗

Routine histological compared with immunohistological examination of bone marrow trephine biopsy specimens in disseminated neuroblastoma.

The ability to detect infiltration in bone marrow biopsy specimens from patients with disseminated neuroblastoma was assessed by immunohistological and routine histological methods. Frozen cores from 33 staging procedures were tested with UJ13A and UJ127.11. Immunopositive tumour cells were found in 10 of 17 staging procedures in which tumour was detectable by routine histological methods. Positive cells or stromal material were also found in eight of 12 staging procedures in which distorted architecture and fibrosis, but no obvious tumour, had been noted. Paraffin wax embedded cores from 29 of the same staging procedures were tested with antibodies against neurone specific enolase and neurofilament. Only a single core reacted with anti-neurofilament antibody. Neurone specific enolase positive cells or stromal material were found in nine of 15 staging procedures in which obvious tumour was detectable. Although these immunohistochemical techniques proved inferior to routine histology in their ability to detect obvious tumour, the demonstration of immunopositive stromal tissue which was not frankly malignant supports the view that distorted, fibrotic marrow may reflect persistence of neuroectodermal tissue and justifies its distinction from normal marrow when reporting the response to treatment.

Adolescent↗

Alpha interferon gene deletions in adults, children and infants with acute lymphoblastic leukemia.

DNA from 76 cases of acute lymphoblastic leukemia (ALL) was tested with a cDNA probe encoding the alpha 2B interferon (IFN) gene transcript. Deletions were found in three of ten pre-B, three of 21 T-cell, four of 22 common and one of 23 null ALL cases. Amongst those with null ALL were 20 infants, most with characteristic translocations, none of whom had deletion of alpha IFN genes. The results confirm that alpha IFN gene deletions may occur without visible abnormalities of chromosome 9p and show that they occur across a wide range of ALL phenotypes. The results suggest that alpha IFN gene deletions may be rare events in null ALL of infants but their incidence and cellular consequences remain unknown.

Adult↗

Lack of response of bone marrow, in vitro, to growth factors in congenital neutropenia.

Severe congenital neutropenia has a poor outlook. In vitro clonogenic assays using recombinant growth factors may improve understanding of the underlying pathogenetic mechanisms and identify those in whom growth factors might be clinically useful. Marrow from a boy with congenital neutropenia was cultured with a variety of recombinant growth factors. The results show that the neutropenia did not result from a lack of myeloid progenitors but that these progenitors could not produce mature neutrophils. Bone marrow transplantation is being considered as the most likely approach to correct neutropenia.

Agranulocytosis↗

Local antibody production and respiratory syncytial virus infection in children with leukaemia.

Children undergoing therapy for acute lymphoblastic leukaemia (ALL) are at increased risk of severe viral respiratory infection, and some find it difficult to terminate virus secretion. This increased severity may result from a defect in the mucosal immune response. To test this hypothesis, nasal immunoglobulin secretion and specific antiviral antibody responses to infection with respiratory syncytial (RS) virus in children with ALL have been compared with those in a normal age-matched comparison group. Children with leukaemia secreted normal levels of IgA and slightly raised IgM levels. IgG levels were depressed. Following RS virus infection, the majority of children with leukaemia secreted normal amounts of IgA and IgG nasal antibody and successfully cleared the virus. However, three of the 13 children studied made poor or undetectable nasal antibody responses, which correlated with their inability to clear the virus.

Antibodies, Viral↗

De novo Ph negative T-cell lymphoblastic leukaemia associated with bcr gene rearrangement.

One case of de novo Ph-negative T-cell acute lymphoblastic leukaemia has been found to have a classical breakpoint cluster region (bcr) rearrangement of the type seen in chronic granulocytic leukaemia. There were no haematological features to suggest a previous chronic phase. This case represents the first report of this rearrangement in Ph negative acute T-lymphoblastic leukaemia at presentation. The implications for various therapeutic options in such patients are discussed.

Adult↗