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Postsplenectomy septic sequelae may be fatal, but the mechanisms are unclear. We hypothesized that peritoneal macrophage (PM phi) antimicrobial function is abnormal following splenectomy and that this may predispose to increased mortality from the fungal pathogen Candida albicans. Study 1 (in vivo): female CD-1 mice were randomized into control (C), laparotomy (L), or laparotomy+splenectomy (L + S) and inoculated with C. albicans (10(7) organisms, ip) and were studied for mortality. Study 2 (in vitro): mice were randomized to C, L, or L + S groups. Twenty-four hours later, PM phi were harvested and studied for their antifungal activity, including percentage PM phi ingestion of C. albicans and vacuolar sealing of C. albicans within PM phi, percentage C. albicans killing, and superoxide anion (O2-) generation, the mechanism by which candida are killed. Results showed decreased phagocytosis and killing of C. albicans in the L + S group (P < 0.05 vs C) and reduced vacuolar sealing (P < 0.05 vs C) but significantly higher O2- release compared to that in other groups (P < 0.05). Mortality in the L + S group from C. albicans sepsis was significantly higher than that in the other groups (60% compared to 20% in the L group and 13% in C, P < 0.02). This may have resulted from L + S-induced defective phagocytosis of C. albicans and depressed C. albicans killing but increased O2- release in response to candida. This discrepancy between decreased killing and increased O2- may result from increased leakage of O2- from more unsealed vacuoles in the L + S group. Thus, L + S may predispose to candida-induced mortality through defective PM phi intracellular candida killing while enhancing the release of O2- extracellularly from unsealed vacuoles, causing tissue injury.

SummaryA case is reported of cervical vertebra plana presenting with many typical features of eosinophilic granuloma, but ultimately proving to be Ewing's sarcoma. The proper diagnostic approach to vertebra plana lesions in children is presented.

Thalidomide has become an important agent in the treatment of myeloma. However, thalidomide induced erectile dysfunction is a serious complication which has received very little attention. In our hematology department, 6 out of 11 male patients developed erectile dysfunction (grade 3 in 5/6) within 4 weeks of starting thalidomide. Our results suggest that thalidomide induced impotence is a common complication in male hematology patients.

Raised percentage hypochromic red cells (%HRC) were detected at diagnosis in 10 of 34 consecutive patients with low-risk myelodysplastic syndrome (MDS) [refractory anemia (RA) (4/26) and RA with ring sideroblasts (6/8)], all of whom had normal or increased serum ferritin and bone marrow iron stores. Elevated %HRC has persisted in all 10 cases and subsequently developed in another RA patient who later had a complete remission of MDS with normalisation of %HRC after a respiratory tract infection. A strong positive correlation was found between %HRC and erythrocyte zinc protoporphyrin levels in 11 MDS patients tested (p=0.01), suggesting that functional iron deficiency contributes to ineffective erythropoiesis in cases of MDS with raised %HRC. Five of seven patients with elevated %HRC had satisfactory haemoglobin responses to a trial of human recombinant erythropoietin without iron supplementation.

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.

16527 The term Hypereosinophilic syndrome (HES) refers to a heterogeneous group of disorders characterised by marked blood (>1500/cu mm) and tissue eosinophilia resulting in end organ damage.Several visceral complications like cardiomyopathies and cerebrovascular accidents are common. Treatment of HES includes corticosteroids,chemotherapeutic agents (hydroxyurea, cyclophosphamide, vincristine), interferon-alpha. Newer treatment modalities,including tyrosine kinase inhibitors (eg Imatinib) and monoclonal anti IL5 antibodies are now available. We report a case series of patients with HES which demonstrate the variable clinical response to above therapeutic options. Case 1: A 43 year old man who presented with a right lacunar infarct had a eosinophil count of 1200/cu mm. No cause was found. He also had Mitral valve endocarditis. Following initial treatment with Methylprednisolone, the eosinophil count came down to 3500/cu mm, but the eosinophilia persisted. He was given a trial of Imatinib. In 2 months time his eosinophilia resolved. However, he was negative for FILIPI-PDGFRa. Case 2: A 37 year old lady with history of Splenectomy (due to trauma) presented with a eosinophil count of 2660/cu mm. Result of the FILIPI-PDGFRa was equivocal. There was no response to Imatinib. A trial of Prednisolone (1 mg/Kg) was ineffective. She did not respond to Interferon-alpha. However when given a combination of Prednisolone (0.5 mg/kg) and Interferon-alfa, her counts were normal in a month. Case 3: A 63 year old man with history of Atrial Fibrillation had persistent eosinophilia (>1500/cu mm). His serum Tryptase was raised but he was negative for FILIPI-PDGFRa. A trial of Imatinib failed He responded partially to high dose of Hydroxyurea. Prednisolone, Interferon-alpha or a combination of both are future options for him. Case 4: A 80 year old man with background history of Alzheimer’s Disease was found to have persistent unexplained eosinophilia. He had no complains of joint symptoms but his ANF was positive in low titres. A bone marrow showed no evidence of lymphoma or a myeloproliferative disorder. Karyotype was normal. He was given a trial of Hydroxyurea to which he responded in two weeks. Thus, response of HES patients to treatment is variable often unpredictable. No significant financial relationships to disclose.

Two cases in which digital arteritis antedated malignant disease are reported. The literature concerning this rare association is reviewed. Cryoimmunoglobulinaemia is suggested as a possible mechanism for digital arteritis.

Bronchial hyperresponsiveness can be produced experimentally after inhalation of numerous nonimmunospecific stimuli; our objective was to determine whether acrolein, a component of cigarette smoke, could increase bronchial reactivity to intravenously administered acetylcholine in guinea pigs. Bronchial responsiveness was assessed twice before and 1, 2, 6, and 24 h after exposures to less than or equal to 0.01 (sham), 0.31, 0.67, 0.94, or 1.26 parts per million for 2 h (5–7 guinea pigs/group). To examine the possible relationships of responsiveness to inflammatory mediator release and cellular infiltration, bronchoalveolar lavage was performed in another 30 guinea pigs before (control) and 0, 1, 2, 6, or 24 h after exposures. Pulmonary resistance was increased immediately after exposure (5 min) and returned to control values within 30–60 min. Increased bronchial responsiveness was evident within 1 h and became maximal 2–4 h after exposure. The acetylcholine dose necessary to double resistance decreased from 104.2 +/- 7.3 to 79.6 +/- 15.9 at 1 h and was 32.5 +/- 7.9 at 2 h and 32.8 +/- 7.6 micrograms.kg-1 at 6 h. Increases in two eicosanoids, thromboxane B2 (from 167 +/- 21 to 314 +/- 77 pg/ml) and prostaglandin F2 alpha (from 98 +/- 20 to 285 +/- 62 pg/ml) occurred immediately after exposure, whereas an influx of neutrophils occurred 24 h later (from 2.2 +/- 1.2 to 11.3 +/- 3.6%). These temporal relationships suggest that neutrophil infiltration may be a sufficient but not a necessary condition for the onset of bronchial hyperresponsiveness and that injury to cells normally present in the lung are responsible for the mediators thought to influence bronchial responsiveness.

A case report of agnogenic myeloid metaplasia (AMM) is presented in which a patient had a preliminary 18-month period characterized by leukemoid reactions to repeated infections alternating with normal health, throughout which an acquired Pelger-Huet anomaly persisted. This is the only reported case of the anomaly as a premonitory hematologic marker of AMM. The literature concerning the acquired Pelger-Hut anomaly is reviewed.

Acute pulmonary artery occlusion is frequently a dramatic occurrence with a well recognized clinical spectrum. In contrast, a chronic occlusion of the pulmonary artery often awaits post-mortem diagnosis. We present a case diagnosed by chest radiograph. A nine-year-old male presented in August 1976 with areas of ischaemic necrosis of two toes on the left foot for which a lumbar sympathectomy was performed with regression of symptoms which did not recur. Five months later the patient developed acute myeloid leukaemia in the course of which consolidation of the right middle lobe occurred.

A 34-year-old woman developed acute myelogenous leukaemia in the course of pregnancy and, after delivery of a normal baby, developed multiple bilateral breast masses composed of myelogenous tissue.

SummaryA 20-year-old male who required splenectomy following abdominal trauma was subsequently shown to have primary thrombocythaemia. This report illustrates the diagnostic problems associated with a refractory post-splenectomy thrombocytosis, and the therapeutic difficulties in the management of primary thrombocythaemia are discussed.