Eltrombopag for resistant immune thrombocytopenia secondary to chronic lymphocytic leukaemia.

Autoimmune manifestations are a common occurrence with chronic lymphocytic leukaemia (CLL). We describe a case of CLL-associated immune thrombocytopenia (ITP) that had a loss of response to standard treatment for ITP. The thrombopoeitin receptor agonist, eltrombopag, was successfully used preoperatively to increase the platelet count to a safer level, in this instance to facilitate laparoscopic splenectomy.

International Myeloma Working Group recommendations for global myeloma care.

Recent developments have led to remarkable improvements in the assessment and treatment of patients with multiple myeloma (MM). New technologies have become available to precisely evaluate the biology and extent of the disease, including information about cytogenetics and genetic abnormalities, extramedullary manifestations and minimal residual disease. New, more effective drugs have been introduced into clinical practice, which enable clinicians to significantly improve the outcome of patients but also pose new challenges for the prevention and management of their specific side effects. Given these various new options and challenges, it is important to identify the minimal requirements for diagnosis and treatment of patients, as access to the most sophisticated advances may vary depending on local circumstances. Here, we propose the minimal requirements and possible options for diagnosis, monitoring and treatment of patients with multiple myeloma.

Multiple myeloma presenting with a fever of unknown origin and development of thrombotic thrombocytopenic purpura post-bortezomib.

Multiple myeloma rarely presents with a fever of unknown origin and diagnosis may be delayed. We describe a case of myeloma presenting in this way with raised serum-free light chains and TP53 deletion on cytogenetics. The patient developed thrombotic thrombocytopenia purpura (TTP) following bortezomib therapy but recovered spontaneously and was successfully re-challenged. We believe this is only the second case to describe this phenomenon post-bortezomib and the first to rechallenge the patient successfully without further recurrence of TTP. Possible mechanisms for this successful rechallenge are discussed.

Managing HIV. Part 5: Treating secondary outcomes. 5.13 HIV-related lymphoma and other malignancies.

Treating systemic malignancies requires specialist skill and experience, with active treatment often providing the best initial palliation of symptoms. A full management plan involving general practitioner, patient and others is essential at this stage of HIV disease.

Investigation of subjects with abnormal iron studies: role of the hepatic iron index.

AIM: Genetic haemochromatosis is a common disorder resulting in increased iron deposition in the liver and other organs but can be difficult to diagnose. The aim of this study was to assess the diagnostic value of the conventional tests for iron overload (percentage saturation of transferrin, serum ferritin and grading of iron staining on liver biopsy) and compare these with the newer quantitative biochemical measurements of liver iron. METHOD: A retrospective analysis was made of 108 consecutive patients referred for quantitative liver iron measurements. Iron studies were obtained in 66 of the 108 subjects of whom 60 had abnormal screening tests defined as percent saturation of transferrin (> 60%) and/or ferritin > 350 micrograms/L for females and > 450 micrograms/L for males. Based on clinical features, biochemical data and treatment outcome these 60 subjects were classified as either genetic haemochromatosis, nongenetic haemochromatosis or indeterminate. One patient with treated genetic haemochromatosis was excluded from subsequent analysis. RESULTS: Although the serum ferritin (p < 0.002), percentage saturation of transferrin (p < 0.001), histological iron grade (p < 0.0001) were significantly higher in the genetic haemochromatosis than nongenetic haemochromatosis group there was considerable overlap. Similarly for the hepatic iron concentration (HIC) (p < 0.0001) overlap occurred. The hepatic iron index (HIC/age) gave the best separation with only three cases being misclassified. A correlation between the HII and histological iron index (visualised iron score corrected for age) in 15 subjects gave an r value of 0.72. CONCLUSION: Based on this study we feel that in addition to visual grading of iron in liver biopsies, the hepatic iron index is helpful in establishing a diagnosis of genetic haemochromatosis.

Hepatitis C seroprevalence in bone marrow transplant recipients and haemophiliacs.

AIM: To determine the prevalence of antibodies to hepatitis C (anti-HCV) in patients who have undergone bone marrow transplantation in Wellington, prior to the introduction of hepatitis C screening, and to contrast these results with the prevalence of anti-HCV in the Wellington haemophiliac population. METHOD: Serum specimens were obtained from 30 patients who had undergone bone marrow transplantation for the treatment of haematological disorders, and from 29 haemophiliacs. Specimens were analysed using a second generation HCV immunoassay. RESULTS: Exposure to blood products was high in bone marrow transplant recipients with subjects receiving red cells or platelets from an average of 53 donors (range 15-100, SD 23.2) during their transplant procedure. Despite the high usage of blood products, only one of the 30 patients tested was positive for hepatitis C on the basis of second-generation antibody testing. Confirmatory testing in this patient, (anti-HCV immunoblot assay) was negative. In contrast, 26 of 29 (89%) haemophiliac patients tested were positive for anti-HCV. CONCLUSION: Although the infective risk of blood products cannot be underestimated, the risk of patients contracting hepatitis C from multiple single-unit transfusions, prior to the introduction of screening for hepatitis C was low. This contrasts with the high risk of hepatitis C seroconversion in patients exposed to pooled plasma products.

A complex chromosome rearrangement forms the BCR-ABL fusion gene in leukemic cells with a normal karyotype.

Chromosome in situ hybridization studies showed that the normal karyotype of leukemic cells from a patient with Ph1-negative, BCR-positive chronic myeloid leukemia (CML) concealed a complex t(9;22;20)(q34;q11;p13). The close association of 5'-BCR and 3'-ABL was demonstrated by field inversion gel electrophoresis, and in situ hybridization showed that BCR-ABL was located on the short arm of chromosome 20. Our findings further indicate that chromosome rearrangement is the cause of BCR-ABL gene fusion in leukemic cells that show a normal karyotype. Results from in situ hybridization studies were consistent with formation of the t(9;22;20) by a two step chromosomal rearrangement, but field inversion gel electrophoresis results indicated a more complex rearrangement.

Treatment of hairy cell leukaemia with recombinant alpha interferon.

Eleven patients with progressive hairy cell leukaemia (three nonsplenectomised) were treated with recombinant alpha-2 interferon (Intron-A or Roferon-A) subcutaneously three times per week at a dosage of 3 x 10(6) units. Ten patients completed at least ten weeks of therapy and could be evaluated; one patient died of haemorrhage from severe thrombocytopenia after only three weeks treatment. Nine of the ten patients responded and all of these are regarded as good partial remissions (normalisation of all blood parameters but still discernible hairy cells in the marrow). Responding patients have all been followed for a median of two years and in one case 3 1/2 years since commencement of therapy. The patients are all transfusion independent and free of infection. We conclude that alpha-2 interferon therapy for progressive hairy cell leukaemia is effective therapy in both splenectomised and nonsplenectomised patients.

Transfusion related HIV infection: the Wellington experience.

A case of transfusion-related AIDS is described which is believed to be the first published case to occur in New Zealand in a nonhaemophiliac patient. The human immunodeficiency virus (HIV) positive donor was shown to be the source of infection in five further HIV positive recipients.