Adult T‐cell leukaemia lymphoma (ATLL) is an aggressive disease caused by the human T‐lymphotropic virus 1 (HTLV‐I) with a short survival. Responses to interferon alpha (IFN‐α) and zidovudine (AZT) have been documented but not with long‐term follow‐up. We treated 15 ATLL patients with IFN and AZT. Eleven patients had acute ATLL, two had lymphoma and two smouldering ATLL, with progression. The main features were: organomegaly (14), skin lesions (10), high white blood cell (WBC) count (11) and hypercalcaemia (9). Eleven patients had previously received chemotherapy and one had received an autograft. At the time of the study, seven patients had progressive disease and eight were in partial or complete clinical remission. Responses (PR) lasting 2+ to 44+ months were seen in 67%; 26% did not respond (NR) and one patient was not evaluable. Hypercalcaemia predicted a poor outcome but differences were not significant. Eight of the 15 patients have died 3–41 months from diagnosis. Median survival for the 15 patients was 18 months. Survival of the NR ranged from 4 to 20 months; six PR patients are alive 8–82 months from diagnosis. The differences in survival between NR (median: 6 months) and PR (55% of patients alive at 4 years) were statistically significant (P = 0·002). In conclusion, IFN and AZT improves the outcome of ATLL patients and helps maintain responses.
PurposeAdult T cell leukaemia/lymphoma (ATLL) is a mature (post-thymic) T cell lymphoma associated with human T-lymphotropic virus type 1 (HTLV-1) infection. Survival in aggressive subtypes remains poor and treatment resistance is frequent. Use of zidovudine (ZDV) and interferon-alpha (IFNα) has been associated with improved response rates in small studies and prolonged overall survival in leukaemic ATLL subtypes in a recent meta-analysis.
Patients and methodsWe report the clinico-pathologic characteristics, treatment and outcome of 73
ResultsThe overall response rate ranged from 55% with chemotherapy alone to 81% with combined first line therapy (chemotherapy with concurrent/sequential ZDV/IFNα).Median overall survival (OS) was 9 months: 7.5 months for acute ATLL and 10 months for lymphoma ATLL. Use of ZDV/IFNα at any time prolonged survival in acute (p=0.0007) and lymphoma ATLL (p=0.0004) and was the sole factor associated with reduction in risk of death in aggressive ATLL (hazard ratio 0.23, 95%CI 0.09-0.60, p=0.002). Combined first line therapy prolonged median OS in acute (p=0.0081) and lymphoma ATLL (p=0.001) compared with chemotherapy alone.
ConclusionThese data support the use of low dose ZDV/IFNα with chemotherapy in the first line treatment of acute and lymphoma ATLL.
We report our experience of peripheral blood and bone marrow changes in patients with HIV disease. Abnormalities were most commonly seen in patients with advanced disease. In AIDS group IV patients (CDC classification) anaemia (92%) neutropenia (85%) monocytopenia (75%) and thrombocytopenia (61%) have their highest incidence, the reason being a combination of factors such as infection, myelosuppressive drugs and HIV infection itself. Bone marrow examinations were performed most commonly for microbiological culture (25%) and the investigation of anaemia (16%). Morphological changes in the bone marrow were non‐specific and not pathognomic; however erythroid hypoplasia was found to be a distinctive feature associated with MAI infection. The procedure provided a high yield for microbiological culture, particularly in MAI infection.
Accurate identification of lymph nodes facilitates nodal assessment by size, morphological or MR lymphographic criteria. We compared the MR detection of lymph nodes in patients with pelvic cancers using T2-weighted imaging, and fusion of diffusion-weighted imaging (DWI) and T2-weighted imaging. Twenty patients with pelvic tumours underwent 5-mm axial T2-weighted and DWI (b-values 0-750 s/mm(2)) on a 1.5T system. Fusion images of b = 750 s/mm(2) diffusion-weighted MR and T2-weighted images were created. Two radiologists evaluated in consensus the T2-weighted images and fusion images independently. For each image set, the location and diameter of pelvic nodes were recorded, and nodal visibility was scored using a 4-point scale (0-3). Nodal visualisation was compared using Relative to an Identified Distribution (RIDIT) analysis. The mean RIDIT score describes the probability that a randomly selected node will be better visualised relative to the other image set. One hundred fourteen pelvic nodes (mean 5.9 mm; 2-10 mm) were identified on T2-weighted images and 161 nodes (mean 4.3 mm; 2-10 mm) on fusion images. Using fusion images, 47 additional nodes were detected compared with T2-weighted images alone (eight external iliac, 24 inguinal, 12 obturator, two peri-rectal, one presacral). Nodes detected only on fusion images were 2-9 mm (mean 3.7 mm). Nodal visualisation was better using fusion images compared with T2-weighted images (mean RIDIT score 0.689 vs 0.302). Fusion of diffusion-weighted MR with T2-weighted images improves identification of pelvic lymph nodes compared with T2-weighted images alone. The improved nodal identification may aid treatment planning and further nodal characterisation.
BACKGROUND
Antenatal cases of Bombay‐phenotype (Oh) individuals and hemolytic disease of the fetus and newborn (HDFN) are not well described in the literature. We present two case reports of high‐titer anti‐H in pregnant Oh individuals and their serologic investigation, clinical management, and subsequent outcomes. We describe current published cases detailing pregnancy in Oh individuals, to add to the evidence base for clinical decision making and management of pregnancy.
STUDY DESIGN AND METHODS
We describe two case reports of high‐titer anti‐H in pregnancy in Oh individuals. We summarize published cases to date, to inform clinical decision making and antenatal management in individuals with the Bombay phenotype.
RESULTS
Of the case reports described, neither were affected by HDFN due to anti‐H. Antibody titers were high in both cases (immunoglobulin G titer scores, 512 and 4000, respectively) and would be expected to cause some degree of HDFN, a surprising finding. Regular mean cerebral artery Doppler ultrasound was normal. Patient blood management (PBM) techniques ensured that the patient's hemoglobin (Hb) levels were monitored and maintained. Transfusion intervention was not required, with minimal blood loss recorded at birth in both cases.
CONCLUSION
High‐titer anti‐H in Oh pregnancies may, in rare cases, cause HDFN, but evidence suggests that this may not be the case in all pregnancies. We recommend a multidisciplinary approach, with prompt referral to a fetomaternal medicine unit, combined with PBM strategies, and a planned delivery with the provision of rare‐phenotype units (if available and if indicated) on standby.
Recent research has shown abnormal lipids in acquired immunodeficiency syndrome (AIDS). In human immunodeficiency virus (HIV) infection hypocholesterolaemia, hypertriglyceridaemia and also low high density lipoprotein (HDL)-cholesterol have all been described. In addition, increased dense low density lipoprotein (LDL) particles and also lipoprotein (a) have been observed in some patients. The use of the protease inhibitors has been associated with diabetes mellitus and also features of insulin resistance. This article looks at these lipid abnormalities in detail and discusses possible therapeutic options that may be available, in order to address them.
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