Lupus syndrome with neutropenia following minocycline therapy - a case report.

An 18-year-old patient is described who presented with febrile neutropenia and hepatitis caused by minocycline therapy. This rare complication of minocycline-induced lupus syndrome is discussed here.

Severe skin rash in two consecutive patients treated with 2-chlorodeoxyadenosine for hairy cell leukaemia at a single institution.

Although hairy cell leukaemia was first described 40 years ago, it is only in the last decade that newer therapeutic agents have enabled effective treatment. The purine nucleoside analogue, 2-chlorodeoxyadenosine (2-CdA) is currently considered as first-line therapy with a very high rate of complete remission. Although adverse events with 2-CdA are increasingly recognized, severe cutaneous reactions have been reported rarely. We describe two consecutive patients treated with 2-CdA for hairy cell leukaemia who both suffered extremely severe cutaneous reactions, one of which was life-threatening.

Coexistence of Polycythaemia Vera with Indolent Myeloma in the same Patient.

We describe a patient with polycythaemia vera, who in the course of investigations for polycythaemia was also found to have multiple myeloma. A 75 year old Caucasian male was refered for investigations of a raised haemoglobin from a dermatology clinic. He was attending dermatology clinic for intractable, generalised pruritus for 6 months. Initial investigations confirmed the diagnosis of primary proliferative polycythaemia. Further examination of the bone marrow and trephine bone biopsy revealed an increase in plasma cells with morphological atypia. Serum protein electrophoresis confirmed a monoclonal band and urinary examination revealed excretion of Bence Jones proteins. Immunoperoxidase stains of the trephine biopsy confirmed monoclonal light chain restriction. Coexistence of polycythaemia vera and multiple myeloma is an extremely rare situation.

Acute myeloid leukaemia complicated by anergic tuberculosis.

A case of acute myeloid leukaemia presenting as pyrexia of unknown origin and weight loss with pancytopenia is described. Initial investigations revealed trilineage myelodysplasia which evolved into acute myeloid leukaemia within 2 weeks of presentation. He was commenced on a standard induction regimen consisting of idarubicin, Ara-C and thioguanine. Throughout his hospital stay he remained febrile. In spite of exhaustive investigations no cause for the pyrexia was found nor did he respond to any form of treatment. He died after 9 weeks in hospital. His post-mortem examination revealed widespread disseminated tuberculosis without any reactive inflammatory tissue response or granuloma formation.

Essential thrombocythaemia followed by multiple myeloma in the same patient.

We describe a patient with essential thrombocythaemia who developed multiple myeloma 7 years after an initial diagnosis of essential thrombocythaemia. A 50-year-old white female presented in 1987 with a 2 year history of low backache, painful swollen ankles and burning feet. Her laboratory investigations suggested a diagnosis of essential thrombocythaemia. Initially she was treated with busulphan without much benefit for 6 months. Subsequently she was treated with hydroxyurea with significant relief of her symptoms and reduction in her platelet count. Seven years after her initial presentation she developed bone pains and anaemia with laboratory investigations confirming a diagnosis of multiple myeloma.

Linear IgA dermatosis, coeliac disease, and extraintestinal B cell lymphoma.

Linear IgA dermatosis is a malignancy associated rare bullous disorder similar to dermatitis herpetiformis. Linear IgA dermatosis differs from dermatitis herpetiformis in that the IgA deposits in the epidermal basement membrane are linear rather than granular. A patient is presented with coeliac disease who presented with linear IgA dermatosis and anaemia caused by chronic low grade B cell lymphoma.

Infection in immunocompromised patients with malignant blood disorders in a district general hospital.

The pattern and management of infection in immunocompromised patients over a period of 3 years in a district general hospital has been studied. A total of 222 positive cultures was obtained in 607 episodes of suspected infection all involving patients with malignant blood disorders. Febrile episodes requiring intravenous antibiotics occurred in 248 instances involving 107 patients. The pattern of organisms cultured and the responses to various antibiotic regimes are reported. The costs of antibiotic therapy are considered in the light of the overall response. Of the patients studied, 54 died, infection having a likely causative or contributory part in 21 of them (less than 10% of infective episodes). We conclude that the infective complications of these disorders, particularly in older patients, can be efficiently managed in a district general hospital when full supportive facilities are available.

Factor V inhibitor in thrombosis.

A 68-year-old previously well woman developed sudden onset of limb gangrene in association with liver dysfunction. An immediately acting inhibitor to factor V with some of the features of lupus anticoagulant was demonstrated. The patient required limb amputation within 2 weeks and activity of the anticoagulant seemed to be on the decline 6 months later.

Two-colour immunoenzymatic technique using sequential staining by APAAP to evaluate two cell antigens.

AIMS: To extend the alkaline phosphatase-antialkaline phosphatase (APAAP) immunoenzyme single stain method to a more generally applicable double stain technique. This will allow two primary antibodies of the same isotype of IgG and specifically the nuclear antigen bromodeoxyuridine (BRdU) to be evaluated with a cell surface antigen identifier. METHOD: Sequential applications of the APAAP method showed two antigen sites by different dye couplings to a common alkaline phosphatase substrate, producing blue and red reaction products on the same slide. Antigens on different cell populations as well as those in different compartments of the same cell were analysed. The method allowed a surface antigen monoclonal to be revealed first, using an optimal fixative, before alcohol/gluteraldehyde fixation was used to start the second (BRdU) staining sequence. RESULTS: An analysis of double staining of T lymphocyte subsets (CD4 and CD8) showed no significant difference in the order of application of the primaries (n = 10) and no significant difference from their corresponding single stain results (n = 50), confirming the validity of the technique where antigens are exclusively distributed. Other examples, including antigens distributed in different compartments of the same cell, displayed discrete staining which implied validity. CONCLUSION: Double staining by APAAP with this technique seems to be applicable to those cases where antigens are exclusively distributed and includes cases where different compartments of the same cell are stained. It is especially useful in revealing antigens that require different fixation and preparation--that is DNA incorporated BRdU with a surface antigen. But it does seem to have a limited ability to produce a dual colour at a common site.

Sea-blue histiocytes and Gaucher cells in bone marrow of patients with chronic myeloid leukaemia.

A retrospective study of 211 bone marrow aspirates from patients with chronic myeloid leukaemia (CML) was undertaken to assess the incidence of Gaucher cells and sea-blue histiocytes. A significant correlation between the presence of these cells and prolonged survival was seen. Such storage histiocytes occurred most often during periods of relapsed chronic phase. This study shows that Gaucher cells and sea blue histiocytes are a common feature of CML and that their accumulation seems to be associated with a prolonged increase in leucocyte turnover.

The diagnosis of lupus anticoagulants by the activated partial thromboplastin time--the central role of phosphatidyl serine.

Liposomes of pure phospholipids were used in a modified APTT test system and the role of phosphatidyl serine (PS) in determining the sensitivity of the test system to the presence of lupus anticoagulants was assessed. Six consecutive patients with lupus anticoagulants and seven haemophiliacs with anticoagulants directed at specific coagulation factors, were studied. Increasing the concentration of phospholipid in the test system markedly reduced the sensitivity to lupus anticoagulants but had marginal effect on the specific factor inhibitors. The same effect was achieved when the content of PS alone was increased in a vehicle liposome of constant composition. The results suggest that the lupus anticoagulants can best be detected by a screening method using an APTT test with a reagent of low PS content. The use of a reagent rich in PS will largely abolish the lupus anticoagulant's effect on the APTT. An approach using the two different types of reagent may facilitate differentiation of lupus inhibitors from other types of anticoagulant.

Aplastic anaemia and polyneuropathy.

We report a previously undocumented association between aplastic anaemia and acute idiopathic polyneuropathy and speculate that it was due to an antibody cross-reacting with haematopoietic stem cells and myelin.