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Immunological studies were performed on a group of 44 haemophilia A and 15 haemophilia B patients who were treated exclusively with blood products manufactured by the Scottish National Blood Transfusion Service (SNBTS). All patients were HIV seronegative throughout the study. Of the haemophilia A patients 14 (32%) had CD4+ lymphocyte subset counts less than or equal to 0.5 x 10(9)/l, compared with one (6%) haemophilia B patient and four (8%) controls. The percentage of activated T cells was greater than 5% in 19/33 (57%) with haemophilia A, 5/9 (55%) haemophilia B and 14/50 (28%) of control subjects. beta 2 microglobulin values greater than or equal to 2.0 mg/l were observed in 19 (43%) haemophilia A and four (26%) haemophilia B patients, compared with one (2%) control. No significant increases in serum interleukin-2 receptor concentrations were observed in 15 haemophilia A and one haemophilia B patients. Significantly elevated levels of IgG, IgM and IgA were observed in the haemophilia A group, but elevation of immunoglobulins was restricted to the IgG class in the haemophilia B group. Of the haemophilia A patients 16/30 (53%) and 6/11 (54%) haemophilia B patients had depression of cell-mediated immunity (CMI) as assessed by delayed-type hypersensitivity responses to intradermally injected recall antigens. There was no correlation between factor VIII or factor IX usage and changes in lymphocyte subsets, beta 2 microglobulin, and immunoglobulin levels. There was, however, a strong correlation between annual factor VIII usage and the degree of depression of CMI for those with haemophilia A but not for those with haemophilia B. No correlation between alterations in the immune parameters and disturbance of liver function tests was observed in either haemophilia A or haemophilia B patients. We conclude that alloantigen or non-HIV viral exposure due to repeated administration of factor concentrates brings about alterations in the immune response, and that these changes are more marked following exposure to intermediate purity factor VIII compared with factor IX concentrate.

Lymphomatoid granulomatosis (LG) is a rare T cell rich, B cell non-Hodgkin's lymphoma which is difficult to diagnose. We present a patient with LG who demonstrated many of the difficulties in diagnosis and highlighted the importance of reviewing the diagnosis if treatment does not have the anticipated effect.A 49 year old male smoker presented to another hospital with a 3 month history of weight loss and sweats. A chest radiograph showed a mass in the left lower lobe and bronchoscopic examination revealed inflammation involving the left lower lobe bronchus. Washings were negative for organisms and malignancy. A computed tomographic (CT) scan showed a cavitating mass in the left lower lobe with multiple smaller opacities throughout both lung fields. Percutaneous fine needle aspiration of the mass showed inflammation with necrotic debris. He was discharged but presented 1 week later with fever and rigors and was transferred to this hospital.On arrival he had a cough productive of copious, foul smelling, purulent sputum. His temperature was 40˚C. There was no lymphadenopathy or hepatosplenomegaly. Neurological examination was normal. C-reactive protein (CRP) was 59 mg/l. Blood, urine and sputum cultures, white cell count, serum angiotensin converting enzyme, P-and C-ANCA, autoantibody screen, complement, test for HIV, viral screening, and brucella titres were all negative or normal. A repeat bronchoscopic examination was non-diagnostic. A repeat CT scan showed a 7 cm cavitating lesion in the left lower lobe with an air/fluid level and minor ''inflammatory changes'' in the right lung.Empirical treatment for a suspected lung abscess was commenced using intravenous benzylpenicillin, gentamicin, and metronidazole. Over the next 21 days his pyrexia settled, sputum cleared, and the CRP level normalised. The chest radiograph remained unchanged. Four weeks later his purulent sputum, fever, and raised CRP level recurred. Open lung biopsy was considered, but the thoracic surgical team declined because of the evidence for active infection. Antibacterial therapy was recommenced with intravenous cefotaxime and metronidazole. As before, there was a good clinical response but without radiological resolution. Further symptomatic relapse occurred once the drugs were withdrawn. Seven months after initial presentation he deteriorated further. A CT scan showed the left lower lobe cavity unchanged but there were now numerous nodular densities throughout the right lung (fig 1). Repeat bronchial biopsies showed abnormal lymphoid tissue characterised by a polymorphous lymphoid infiltrate containing scattered enlarged blast-like cells. These stained positively for CD20, a B cell marker, and immunoglobulin rearrangement studies showed clonality. These findings suggested lymphomatoid granulomatosis.He was treated with methylprednisolone, vinblastine, and cyclophosphamide. Within 2 weeks his dyspnoea, fever, and right sided radiological abnormalities resolved. The left lower lobe lesion reduced by 40% after three cycles of chemotherapy. Howev...

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Ains-To investigate the incidence of functional hyposplenism in a group of patients who had undergone allogeneic bone marrow transplantation (BMT). Methods-Splenic function was assessed by counting the number of gluteraldehyde fixed red blood cells containing pits or indentations as examined by interference phase microscopy. Normal values are <2% whereas splenectomy patients have values of 25 to 40%. Results-Twenty eight BMT recipients (17 men, 11 women) were studied at varying periods post-transplant and the results compared with 20 healthy volunteers and 10 patients who had undergone splenectomy or had splenic atrophy because of haematological conditions. Ofthe 28 BMT recipients, one had undergone a prior splenectomy; ofthe remaining 27 patients, four (15%) had evidence of functional hyposplenism with between 5 0 and 34 0% pitted cells. Of these four patients, one had active extensive chronic graft versus host disease (GvHD) which has been previously reported to be associated with functional hyposplenism following transplantation. Only one of the four patients had peripheral blood red cell changes typical of hyposplenism. Conclusion-These results confirm that extensive chronic GvHD is associated with hyposplenism. Intermediate degrees of functional hyposplenism may also occur following BMT in the absence of chronic GvHD and in the absence of haematological features of hyposplenism on routine blood films. This may be of significance in mediating the susceptibility to infection with encapsulating bacteria seen following allogeneic BMT. (J Clin Pathol 1995;48:257-259)

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IL-6 blockade in a case resistant to TNF neutralization should prompt a second diagnostic workup. Rheumatology key message. Neutralization of IL-6 by tocilizumab induces complete clinical remission and controls the growth of popliteal angiomatoid malignant fibrous histiocytoma.