Hodgkin lymphoma in a patient with chronic lymphocytic leukaemia--a rare presentation of Richter's transformation.

Richter's transformation of chronic lymphocytic leukaemia (CLL) to high-grade B-cell Non-Hodgkin lymphoma occurs in < 5% of CLL cases. Transformation of CLL to Hodgkin Lymphoma is a much rarer event and here we describe a patient who developed Richter's transformation into a Hodgkin Lymphoma presenting as rapidly progressive hepatosplenomegaly.

The impact of service user's suicide on mental health professionals.

OBJECTIVES: Our principle objective was to examine the personal and professional impact of service user (SU) suicide on mental health professionals (MHPs). We also wished to explore putative demographic or clinical factors relating to SUs or MPHs that could influence the impact of SU suicide for MHPs and explore factors MHPs report as helpful in reducing distress following SU suicide. METHODS: A mixed-method questionnaire with quantitative and thematic analysis was utilised. RESULTS: Quantitative data indicated SU suicide was associated with personal and professional distress with sadness (79.5%), shock (74.5%) and surprise (68.7%) particularly evident with these phenomena lasting less than a year for more than 90% of MHPs. MHPs also reported guilt, reduced self-confidence and a fear of negative publicity. Thematic analysis indicated that some MHPs had greater expertise when addressing SU suicidal ideation and in supporting colleagues after experiencing a SU suicide. Only 17.7% of MHPs were offered formal support following SU suicide. CONCLUSION: SU suicide impacts MHPs personally and professionally in both a positive and negative fashion. A culture and clear pathway of formal support for MHPs to ascertain the most appropriate individualised support dependent on the distress they experience following SU suicide would be optimal.

Enzyme enhanced solid-state fermentation of kenaf core fiber for storage and pretreatment.

Kenaf is an annual fiber crop adaptable to a wide range of climates and soil types. This study investigated the use of kenaf core fiber as a feedstock for enzyme-enhanced fermentation. Triplicate kenaf core fiber samples were treated with enzymes having cellulase:hemicellulase activity ratios of 0:1, 0.015:1, 0.45:1, and 2.54:1 at a rate of 5010 IU/kg dry matter hemicellulase activity, vacuum-sealed, and incubated at 37 degrees C for 21 d. Samples were analyzed for pH, water soluble carbohydrates, organic acids, and hemicellulose and cellulose concentrations. All treatments produced a pH less than 4.0, which is sufficient for stable storage. Treatments with 2.54:1 and 0.45:1 produced the highest water soluble carbohydrate and lactic acid concentrations. Enzymes with no or low cellulase activity produced results similar to the control. Utilizing enzyme mixtures with high cellulase activity is an effective pretreatment method for ensiled kenaf core fiber.

Autologous stem cell transplantation in myeloma: the St James's Hospital experience, 1997-2003.

BACKGROUND: High-dose treatment with autologous stem cell transplantation (ASCT) has become the standard of care for patients with myeloma below the age of 65 years. AIMS: We report an audit of 60 patients (median age: 52.5 years) who underwent ASCT in the National Bone Marrow Transplant centre in St James's Hospital in Dublin between 1997 and 2003 inclusive. METHODS: Clinical and laboratory data were retrieved from patient medical records and hospital information management systems. RESULTS: Thirty-six patients had IgG, 11 IgA, 1 IgD, 9 light chain and 3 non-secretory MM. Fifty-seven (95%) patients received anthracycline-corticosteroid combination chemotherapy prior to autografting. There was no transplant-related mortality (TRM). Complete (CR) and Partial Responses (PR) were seen in 16 (29.6%) and 29 (53.7%) of those evaluable (n = 54 (90%)). The actuarial Progression-Free (PFS) and Overall Survival (OS) rates at five years are 13% and 55% respectively. CONCLUSION: Centre outcome is comparable to published international series and supports the use of ASCT in the treatment of this malignancy.

Chronic myeloid leukemia and acromegaly in POEMS syndrome.

We describe an acromegalic patient who has developed both chronic myeloid leukemia (CML) and a plasma cell dyscrasia. However, as the patient also has neuropathy, hyperhydrosis and other endocrine disorders, he appears to have an atypical form of POEMS syndrome.

Successful use of topical retinoic acid in severe dry eye due to chronic graft-versus-host disease.

Topical retinoic acid has proved to be of variable benefit in a number of dry eye disorders of disparate aetiology, in which squamous metaplasia with keratinization of ocular epithelium is present. Its exact role in patients with dry eye however remains in dispute. We describe a case of severe dry eye due to chronic graft-versus-host disease, which was refractory to conventional therapy but which responded remarkably to topical retinoic acid with reversal of conjunctival keratinization and marked resolution of symptoms.

Dermatoglyphic fluctuating asymmetry and atypical handedness in schizophrenia.

Atypical handedness and dermatoglyphic abnormalities are hypothesized to reflect a neurodevelopmental disturbance in schizophrenia. Developmental instability, indexed by dermatoglyphic fluctuating asymmetry (FA), reflects the degree to which an individual's ontogenetic program is maintained and provides a useful framework in which to consider atypical handedness in schizophrenia. Thirty patients diagnosed with schizophrenia were compared with 37 matched healthy controls on levels of dermatoglyphic FA, a demonstration task determining hand preference and a test of relative hand skill. Multivariate analyses established that patients demonstrated greater FA and more atypical hand skill compared with controls. In patients, but not in controls, there was a strong positive association between a measure of FA and a measure of atypical hand skill, suggesting that these markers of neurodevelopmental disturbance are related in schizophrenia. On a measure of hand preference, patients were more likely than controls to be classified as mixed handed than either right or left handed. Results from the present study support the conjecture of greater developmental instability in schizophrenia affecting neurodevelopmental processes, including those conferring manual dominance.

POEMS syndrome and Waldenström's macroglobulinaemia.

A 58 year old man presented with a three year history of impotence, night sweats and ankle swelling. On examination, the patient fulfilled the diagnostic criteria for POEMS syndrome, but was unusual in that he also had underlying Waldenström's macroglobulinaemia with IgM kappa paraproteinaemia. The patient was treated with intermittent chlorambucil and made a good recovery. POEMS syndrome has been described in association with osteosclerotic myeloma and Castleman's disease. The paraprotein involved is usually IgG or IgA with lambda light chains. This case indicates that the presence of lambda light chains is not essential for the pathogenesis of POEMS syndrome. It also emphasises the diversity of plasma cell dyscrasias that can manifest as POEMS syndrome.

Lymphoma associated bone marrow necrosis with raised anticardiolipin antibody.

A case of high grade B cell lymphoma presented with bone marrow necrosis, followed by development of extensive marrow fibrosis, the evolution of which was documented by serial magnetic resonance imaging and bone marrow trephine histology. A markedly raised anticardiolipin antibody titre at diagnosis suggests that lymphoma associated antiphospholipid syndrome may have contributed to the aetiology of the bone marrow necrosis.

Interleukin-4 and tumour necrosis factor-alpha produce non isotype specific partial differentiation of peripheral blood B-cells in myeloma.

In a previous study, culture of peripheral blood mononuclear cells (PBMC) from myeloma patients with interleukin(IL)-4 and tumour necrosis factor(TNF)-alpha resulted in the appearance of clonal plasma cells, thus suggesting the presence of circulating myeloma cell precursors in the peripheral blood. Using the same cytokine combination, we cultured PBMC and purified peripheral blood B-cells from myeloma patients. In nearly all cases, partial differentiation of B-cells occurred but, similarly to results for normal controls, both kappa and lambda light chain (L.C.) cytoplasmic positive lymphoid and lymphoplasmacytoid cells were detected rather than clonal plasma cells. These results suggest that IL-4 and TNF-alpha cause partial differentiation of residual normal polyclonal B-cells rather than of circulating myeloma cell precursors in the peripheral blood of myeloma patients.

Identification and treatment of anaemia in older patients.

Anaemia in elderly patients should never be regarded as a normal physiological response to aging. Underlying causes must be investigated and treated in a similar manner to that used in younger adults. In addition to a thorough history and physical examination, basic investigations such as red cell indices and morphology, reticulocyte count, haematinic assays and occasionally bone marrow examination, will detect the underlying pathology in most cases. Anaemia may be classified, according to red blood cell mean corpuscular volume, into microcytic, macrocytic and normocytic types. Anaemia with an absolute reticulocytosis is due either to acute blood loss or haemolysis. Other anaemias, more frequently encountered in elderly patients, are hypoproliferative, and reflect depressed marrow production or impaired erythroid maturation. Examples include anaemia of chronic disease and iron deficiency and, less commonly, megaloblastic anaemia and anaemia due to primary bone marrow failure. The treatment of anaemia should aim to correct the underlying cause of the disorder and/or to improve the quality of the blood, e.g. by haematinic replacement therapy. Recombinant human erythropoietin has revolutionised the treatment of anaemia associated with chronic renal failure, while its role in other anaemias is currently under investigation. Regular blood transfusion may be required for some elderly patients with chronic anaemia. However, the attendant risks of this procedure, such as iron overload and viral hepatitis transmission, must be considered.

The distribution of brominated long-chain fatty acids in sponge and symbiont cell types from the tropical marine sponge Amphimedon terpenensis.

The tropical marine sponge Amphimedon terpenensis (family Niphatidae, order Haplosclerida) has previously been shown to possess unusual lipids, including unusual fatty acids. The biosynthetic origin of these fatty acids is of interest as the sponge supports a significant population of eubacterial and cyanobacterial symbionts. The total fatty acid composition of the sponge was analyzed by gas chromatography/mass spectrometry of the methyl esters. Among the most abundant of the fatty acids in intact tissue were 16:0, 18:0 and 3,7,11,15-tetramethyl-hexadecanoic (phytanic) acid. In addition, three brominated fatty acids, (5E,9Z)-6-bromo-5,9-tetracosadienoic acid (24:2Br), (5E,9Z)-6-bromo-5,9-pentacosadienoic acid (25:2Br) and (5E,9Z)-6-bromo-5,9-hexacosadienoic acid (26:2Br) were also present. The three brominated fatty acids, together with phytanic acid, were isolated from both ectosomal (superficial) and choanosomal (internal) regions of the sponge. Analysis of extracts prepared from sponge/symbiont cells, partitioned by density gradient centrifugation on Ficoll, indicated that phytanic acid and the three brominated fatty acids were associated with sponge cells only. Further, a fatty acid methyl ester sample from intact tissue of A. terpenensis was partitioned according to phospholipid class, and the brominated fatty acids were shown to be associated with the phosphatidylserine and phosphatidylethanolamine fractions that are commonly present in marine sponge lipids. The phosphatidylcholine and phosphatidylglycerol fractions were rich in the relatively shorter chain fatty acids (16:0 and 18:0). The association of brominated long-chain fatty acids (LCFA) with sponge cells has been confirmed. The findings allow comment on the use of fatty acid profiles in chemotaxonomy and permit further interpretation of LCFA biosynthetic pathways in sponges.(ABSTRACT TRUNCATED AT 250 WORDS)

Isolation of brominated long-chain fatty acids from the phospholipids of the tropical marine sponge Amphimedon terpenensis.

Preliminary investigation of the phospholipid fatty acid composition of the tropical marine sponge Amphimedon terpenensis by gas chromatography/mass spectrometry revealed the presence of some novel brominated fatty acids. Two new brominated fatty acids, (5E, 9Z)-6-bromo-5,9-tetracosadienoic acid (2a) and (5E, 9Z)-6-bromo-5,9-pentacosadienoic acid (3a) were subsequently isolated from a chloroform/methanol (3:1, vol/vol) extract of the sponge and characterized as their methyl esters 2b and 3b. The known brominated fatty acid (5E, 9Z)-6-bromo-5,9-hexacosadienoic acid (4a) was also isolated. The new fatty acid methyl esters were confirmed as brominated delta 5,9 acid derivatives by chemical ionization mass spectrometry. The position of the bromine substituent was determined to be C-6 by nuclear magnetic resonance techniques while the stereochemistry of the two double bonds was deduced by nuclear Overhauser enhancement difference spectroscopy. The biosynthetic implications of the co-occurrence of the three brominated acids are discussed.

The distribution of lipids and sterols in cell types from the marine sponge Pseudaxinyssa sp.

The sponge Pseudaxinyssa sp., unique in sterol and fatty acid composition, was cellularly dissected into fractions enriched in each of the major cell types present in the sponge: microbial symbionts (cyanobacteria), small sponge cells (pinacocytes and choanocytes), and large sponge cells (archeocytes and cyanophytes). Three phototrophic microbial symbionts were also isolated from the cell fractions and grown in culture. An unsymmetrical distribution of fatty acids and sterols was observed for the sponge cells: small cells contained larger quantities of long chain fatty acids (greater than C24) and smaller quantities of sterols than were present in the larger sponge cells. Moreover, the rare sterols 24-isopropylcholesterol predominated in the smaller sponge cells, whereas its 22-dehydro analog predominated in the larger sponge cells. Long chain fatty acids and sterols were not detected in the cultured microbial symbionts. This constitutes the first report of lipid variability according to cell type for this most primitive group of Metazoa.

Australian deliberations on access to its terrestrial and marine biodiversity.

The predominantly developed country business principle that the natural resource is effectively free, or of very low monetary value, has been significantly challenged in recent years, not only through the recognition of the accelerated rate of depletion of native forest resources and of the space and food demands of increasing populations, but also through international conventions which deal with a wide range of topics from the rights of indigenous people to the Law of the Sea Convention. Australia, classified as a developed country, but located in a geographic region of many developing countries, has, in the past 25 years, demonstrated particular concern for the rights of the people of those countries, as well as for the rights of indigenous people of Australia. The practical international aspects were clearly exemplified in the time, from 1985, when the Australian Institute of Marine Science (AIMS) negotiated, within the National Cancer Institute (NCI) contract, that collections of biological samples in developing countries would be accompanied by an agreement to provide benefits arising from field work, and from any commercial product developments, to those countries. Australia, as a signatory to the Convention on Biological Diversity (Appendix I), continues to analyze the challenge presented by the need to freely exchange genetic resources of common value, e.g. food crops, while insuring an appropriate reward to developing and developed countries, should discoveries be made from their biological resources, which lead directly or indirectly, to high value commercial non-food products. The Prime Minister's Coordinating Committee on Science and Technology established a special working group to recommend on access to Australia's biodiversity. The report arising from the study, and other related issues, are discussed.

Adjunctive treatment of compartment syndrome with hyperbaric oxygen.

Trauma-induced compartment syndrome and other acute traumatic peripheral ischemias have been effectively treated with hyperbaric oxygen therapy. We describe a case of compartment syndrome associated with an acute exertional injury. After surgical decompression, hyperbaric oxygen therapy reduced edema and improved tissue viability. The mechanisms of hyperbaric oxygen applicable to the pathophysiology of compartment syndrome are described. We believe that hyperbaric oxygen is a useful intervention in the management of compartment syndrome.

B19 parvovirus infection causing aplastic crisis in 3 out of 5 family members with hereditary spherocytosis.

B19 parvovirus infection is a principal cause of transient bone marrow suppression in patients with chronic haemolytic anaemias. Acute infection is described in a family of five individuals with hereditary spherocytosis; three of them developed marked hypoplastic anaemia.