Pulmonary vasculitis as an extraintestinal manifestation in ulcerative coliltis

Pulmonary complications are rarely reported in association with ulcerative colitis. Our patient had ulcerative colitis, diagnosed three decades earlier. Following a relapse of his ulcerative colitis, the patient developed bloodstained sputum. Chest CT-scan showed signs of pulmonary tissue infiltration indicating pulmonary vasculitis. No causative agent for this clinical condition was found. Testing for ANCA showed the vasculitis to most likely be secondary to his ulcerative colitis. The lung lesions responded to corticosteroid therapy within a week, and the following chest CT-scan was normal.     

Morvan's fibrillary chorea: a paraneoplastic manifestation of thymoma

Morvan's fibrillary chorea is a rare disease characterised by symptoms which include neuromyotonia, cramping, weakness, pruritus, hyperhidrosis, insomnia, and delirium. The first case of Morvan's fibrillary chorea to be associated with clinical manifestations of myasthenia gravis with thymoma, psoriasis, and atopic dermatitis is reported. Muscle histopathology disclosed chronic denervation and myopathic changes and in vitro electrophysiology demonstrated both presynaptic and postsynaptic defects in neuromuscular transmission. Serum antibodies to acetylcholine receptors, titin, N-type calcium channels, and voltage gated potassium channels were detected. Plasmapheresis, thymectomy, and long term immunosuppression induced a dramatic resolution of symptoms. The association of thymoma with other autoimmune disorders and autoantibodies, and prolonged and sustained remission with chronic immunosuppression, place Morvan's fibrillary chorea on the range of neurological diseases arising as a paraneoplastic complication of cortical thymomas.     

Epilepsia partialis continua: a new manifestation of anti-Hu-associated paraneoplastic encephalomyelitis

We report on 3 anti-Hu-positive patients who presented with clinical and electroencephalographic (EEG) features of epilepsia partialis continua (EPC). Two of the patients had an associated small cell carcinoma. Magnetic resonance imaging (MRI) disclosed a hyperintense nonenhancing focal lesion in T2-weighted images in the sensorimotor area in 2 patients. Histopathological analysis of the lesion revealed inflammatory infiltrates and neuronal cell loss. In the patient who had a postmortem study, these neuropathological changes were not observed in other areas of the nervous system. This study emphasizes that the possibility of an anti-Hu-associated paraneoplastic disorder must be considered in patients with cortical encephalitis presenting with EPC when a brain tumor can be excluded.     

Trisomy 5 in two cases of acute monocytic leukemia with hyperdiploid clones

Although numerical chromosomal aberrations are commonly seen in acute myeloid leukemia (AML), trisomy 5 (+ 5) is very rarely detected. We report two patients, both of whom suffered from acute monocytic leukemia, in which + 5 was found in hyperdiploid clones. A review of the English literature shows 17 additional cases of AML with + 5 in at least one of the abnormal clones, making a total of 19 such cases including ours. Trisomy 5 has been reported in all FAB subtypes of AML except acute promyelocytic leukemia. In the 19 cases identified in this report, + 5 was found in association with other numerical changes (four cases), structural changes (five cases) or both (eight cases). Trisomy 5 as a sole karyotypic abnormality was exceedingly rare (two cases). Its biologic and prognostic significance remains to be determined.     

Intracerebral hematoma as an acute manifestation of intracranial tumors

Sixteen patients with spontaneous intracerebral haematoma due to intracranial tumours are discussed. The total number of patients with intracranial tumour treated in the period concerned was 594, so the incidence of tumour haemorrhage was 2.7%. In most of the patients we found metastatic neoplasm (n = 6) and glioblastoma (n = 3). All patients were acute admissions. Seven were comatose, seven were somnolent, and two patients were alert. In ten patients the haemorrhage represented the first reliable clinical sign. In six patients a tumour disease was known. Fourteen patients were operated on. Six patients died. Extensive neuro-radiological examination is very important, particularly since 30% of these acute intracerebral haemorrhages occur in patients with benign intracranial tumours.     

MT方案治疗急性单核细胞白血病的疗效及其与染色体核型的关系

目的 研究米托蒽醌联合替尼泊苷(MT)方案在急性单核细胞白血病(M5)诱导缓解中的疗效及患者不良反应,并观察疗效与白血病染色体核型的关系.方法 将33例M5患者按治疗史分两组:初治组23例(A组)、DA(柔红霉素联合阿糖胞苷)或HDA(三尖杉酯碱、柔红霉素和阿糖胞苷)1个疗程无效组10例(B组).按核型预后分两组:预后中等组29例(C组),预后不良组4例(D组),均采用MT方案2个疗程诱导缓解,分别统计4组的临床疗效及患者不良反应.结果 MT方案对A、B组的M5诱导完全缓解(CR)率分别为83%(19/23)及60%(6/10),有效率达91%(21/23)及70%(7/10).C、D组CR率分别为83%(24/29)及25%(1/4),有效率为88%(26/29)及50%(2/4),其中复杂核型CR率为0(0/3),非复杂核型的11q23染色体异常患者一次化疗达CR率100%(4/4).MT方案对M5化疗后白细胞最低点在第(7±3)天出现,为(0.4±0.2)×109/L,白细胞<1×109/L时间达(8±5)d,未见化疗相关死亡病例.结论 MT方案简单有效、较安全,是治疗M5的较佳化疗方案,对1个疗程DA、HDA方案无效者亦可试用.MT方案化疗疗效与核型预后分组有关,对11q23染色体异常的M5患者疗效较好,对复杂核型患者疗效欠佳.     

Soluble L-selectin increases in the cerebrospinal fluid prior to meningeal involvement in children with acute lymphoblastic leukemia

We have treated 19 B-chronic lymphocytic leukaemia (B-CLL) patients with CDA (Leustat, Janssen-Cilag). Four patients developed severe autoimmune haemolytic anaemia, and 2 of these had severe reticulocytopenia due to red cell aplasia/hypoplasia. Two patients died as a complication of the haemolysis one during the primary episode, with a clinical course suggestive of transfusion associated graft-versus-host disease (taGVHD), and one following a relapse of haemolysis. The onset of haemolysis occurs within 4 cycles of CDA therapy and is temporally related to the T-lymphocyte nadir induced by CDA. The presence of a positive DAT prior to therapy in 3 of 4 patients developing haemolysis suggests that the CDA induced T-lymphocytopenia may exacerbate the tendency of certain CLL patients to autoimmune haemolysis.     

Persistent erythema infectiosum-like rash as a prodrome of acute lymphocytic leukemia

A 7-year-old boy had erythema infectiosum with typical manifestations. Over more than 40 days, these lesions showed no sign of regression, and chronic anemia became progressively more severe. Electron microscopic examination performed 20 days after onset showed abnormal Sezary-like lymphocytes. Bone marrow biopsy, which was performed to evaluate the anemia, was consistent with acute lymphocytic leukemia. Persistent parvovirus B19 infection may be connected with immunosuppression. Therefore, early electron microscopic study and bone marrow biopsy may be helpful for early diagnosis of hematologic malignancies.     

Cutaneous vasculitis, mixed cryoglobulinemia in a patient with non-secreting monolateral pheochromocytoma. A likely paraneoplastic syndrome

We describe a patient who presented with purpuric lesions over his legs, asthenia and arthralgias. The patient was diagnosed as having cryoglobulinemia and no secreting monolateral pheochromocytoma. Two months after the removal of the tumour, the vasculitic lesions disappeared but the cryoglobulins were still present in the patient's serum. The cryoglobulins, which varied from 30 mg/dl to 60 mg/dl, persisted even during the following year. We consider this case noteworthy because the association among leukocytoclastic vasculitis, cryoglobulinemia and no secreting monolateral pheochromocytoma has not been previously described.     

Hypersensitivity as a cause of dermatologic and vaginal moniliasis resistant to topical therapy

The finding of multiple respiratory or skin allergies together with a high rate of improvement by desensitization leads the author to conclude that allergy is a predisposing factor for the chronicity or recurrence of vaginal or dermatologic moniliasis. Desensitization with Candida albicans antigen was the mainstay in the treatment of resistant dermatologic or vaginal moniliasis and the sole form of therapy in cases of nail involvement with deep seated lesions out of the reach of topical agents.     

Severe acute liver failure as the initial manifestation of haematological malignancy

Acute liver failure is rarely secondary to lymphoma or leukaemia and it is extremely uncommon as the initial presentation of malignancy. We report a case of a young adult patient with severe acute liver failure referred for liver transplant, in which a Burkitt acute lymphoblastic leukaemia was diagnosed by bone marrow examination. A complete recovery and long remission were obtained with chemotherapy.     

Headache as a manifestation of myocardial infarction

A 33-year-old pregnant woman at 26 weeks gestation, who had a history of bipolar mood disorder, type I, was admitted to the hospital for hypomania and poorly controlled diabetes mellitus. The patient had had her first episode of affective illness at age 28, after the birth of her second child. After an initial postpartum depression, she had cycled into a manic state. She had subsequently been hospitalized seven times for acute mania. A combination of valproate and chlorpromazine had proven effective in managing most of her manic episodes, while her two most severe episodes had been successfully managed with bilateral ECT.     

Congenital leukemia cutis: an unusual manifestation of a rare disease

This paper discusses a case of congenital leukemia cutis of lymphoblastic type presenting as a solitary frontonasal tumor. The presentation is unusual when compared with other reported cases of neonatal leukemia and represents the only reported case with comparable presenting features to the authors' knowledge. The differential diagnosis with other frontonasal tumors is discussed.     

Mixed cryoglobulinemia as a model of systemic vasculitis

Leukocytoclastic vasculitis is the dominant lesion of mixed cryoglobulinemia (MC). The high prevalence of antibodies to hepatitis C virus (HCV) in association with the higher concentration of HCV RNA genomic sequences in the cryoglobulins suggests a close relationship between MC and HCV infection and strongly supports the view that this virus plays a key role in causing vascular damage. Analysis of the composition of immune complexes (ICs) provides evidence that cryoglobulins include virions mostly bound to IgG that is specifically reactive with HCV-related proteins, which in turn are crosslinked by monoclonal IgM with rheumatoid factor (RF) activity, frequently bearing the WA crossidiotype (XId). This structure is similar (if not identical) to that of circulating ICs from HCV-infected patients without cryoglobulins, suggesting that the virus may be directly responsible for the production of WA RF. Evidence for the role of circulating cryoproteins in the pathogenesis of cutaneous and renal vasculitis stems from the demonstration of HCV-related proteins and/or HCV RNA genomic sequences in the vessel wall of patients with MC. Our data indicate that endothelial cells are fully susceptible to infection by and replication of HCV, and support the contention that they serve as sufficient targets for the binding of HCV proteins expressed on the cell surface to serum immunoglobulins. The in situ demonstration of IgM RF WA XId adds further evidence that RF of the WA group participates in the development of vasculitis and probably stabilizes the binding of IgG antibodies. Lymphocytes may be crucial in the infection of endothelial cells by acting as a circulating viral reservoir. After encouraging initial results, controlled trials have defined the substantive efficacy of IFN-alpha in the treatment of MC. A response of IFN can be achieved in more than 50% of patients and includes improvement of cutaneous vasculitis and renal function. This clinical response is accompanied by a reduction in hepatitis C viremia, serum cryoglobulin concentration, and IgM RF synthesis. However, almost 80% of responders eventually have a clinical and biochemical relapse. Additional studies are required to improve the outcome and extension of this therapy, define the best candidates, and indicate the situations in which it is needed.     

Integrin signaling to NF-kappa B in monocytic leukemia cells is blocked by activated oncogenes

Integrin-mediated signals play an important but poorly understood role in regulating the growth and behavior of tumor cells. In monocytes and monocytic leukemia cells, integrin-mediated adhesion results in a strong induction of a set of immediate early genes that are characteristic of monocytic differentiation and contain consensus NF-kappa B elements in their 5' regulatory regions. To investigate the role of integrin signaling in control of differentiation in a human monocytic leukemia cell line, THP-1 cells were transiently transfected with an NF-kappa B driven CAT reporter gene. Adhesion to fibronectin or cross-linking of beta1 integrins resulted in an NF-kappa B-dependent induction of CAT activity. To evaluate whether integrin signaling in this system intersects with the Ras signal transduction cascade, THP-1 cells were cotransfected with the NF-kappa B reporter and with plasmids that direct the synthesis of normal or mutant forms of Ras or Raf. We found that Ras or Raf dominant negative mutants did not inhibit integrin-mediated activation of the NF-kappa B-driven reporter. However, cotransfection with activated Ras, or with several other cytoplasmic oncogenes, blocked this process. This suggests that in monocytic leukemia cells, an antagonism exists between the mitogenic signals provided by oncogenes and the signals generated by integrin ligation. This antagonism may play an important role in regulating the balance between proliferation and differentiation in monocytic leukemias.     

Therapeutic approach to acute myeloid leukemia

The treatment of acute myeloid leukemia has evolved considerably over the past decade. While induction therapy appears to have been almost standardized, there continues to be an ongoing debate and controversy regarding the best form of post-remission therapy. Attempts are being made to identify patients at particular risk of relapse to enable appropriate selection of best induction and post-remission therapies. In these respects, cytogenetics are useful. New concepts include the use of growth factors, immunotherapy, MDR modulation and therapies evolving from a better knowledge of the anomalies of the genes that cause leukemia.     

"Refuses to walk" as an ED presentation of acute childhood leukemia

Ovarian stimulation is used to treat female infertility, especially in Black Africa where infertility is considered as shameful. Ovarian stimulation can lead not only to multiple pregnancies but also to severe systemic complications such as the one described in this report. Ovarian stimulation using human menopausal gonadotropin led to ovarian hyperstimulation and multiple organ failure in a 28-year-old Senegalese woman. Symptomatic treatment using corticosteroids, abdominal paracentesis to relieve ascites, and fluid expansion failed. Bilateral ovariectomy was performed resulting in permanent sterility. Ovarian stimulation requires close monitoring by ultrasound visualization and measurement of 17 beta-estradiol to allow early detection of complications.