淋巴结血管内T细胞淋巴瘤1例报道及文献复习

目的　探讨血管内淋巴瘤 (IVL)的临床病理特征。方法　对 1例腹股沟淋巴结IVL临床、病理组织学及免疫表型进行观察分析并复习文献。结果　男性 31岁 ,不明原因高热伴消瘦 5 0天 ,右腹股沟直径 1cm淋巴结 1枚 ,B超示肝脏轻度增大 ,血LDH明显升高伴ESR及转氨酶轻度升高 ,外周血WBC 3 3× 10 7/L ,骨髓像、多种病原及各肿瘤相关抗原检测均无异常。病理活检 :腹股沟淋巴结大部分破坏 ,代之以大量扩张的中小血管 ,腔内充满大量异型淋巴样细胞 ,局部伴管壁、管周浸润并累及结外脂肪组织。瘤细胞免疫表型CD4 5、CD4 5RO、CD3阳性 ,CK、CD6 8、CD79α、CD2 0均阴性 ,血管壁及内皮细胞CD31、CD34阳性。行CHOP化疗后症状缓解 ,现仍在随访中。结论　IVL是一罕见的非霍奇金淋巴瘤 ,好发于中枢神经系统及皮肤 ,其他部位少见 ,绝大数为B细胞型 ,T型罕见 ,以浅表淋巴结活检确诊者尚无报道。临床表现有一定提示性 ,确诊靠组织病理学检查 ,部分病例对化疗敏感 ,但多数病例预后差     

An autopsy case of giant cell myocarditis probably due to a non-steroidal anti-inflammatory drug

An autopsy case of giant cell myocarditis (GCM) in a 74-year-old woman is presented. She suffered from hepatic dysfunction, skin eruption and disseminated intravascular coagulation due to the side-effects of a non-steroidal anti-inflammatory drug. After admission, heart failure progressed rapidly, and the patient died suddenly. At autopsy, her heart was slightly enlarged and the heart muscle was thickened with many small whitish nodules. She was diagnosed with GCM because of the infiltration of multinuclear giant cells, histiocytes, eosinophils and lymphocytes into the heart. We did not find any similar lesions in any other organs. Giant cell myocarditis, the etiology of which is not defined, is a rare disease with unfavorable prognosis. This case suggests the possibility of drug-induced GCM.     

Intravascular large B-cell lymphoma presenting with mass lesions in the central nervous system: a report of five cases

We have encountered five cases of intravascular large B-cell lymphoma (IVL) presenting with central nervous system (CNS) mass lesions during their clinical course. The age of the patients ranged from 50 to 74 years and three patients were male. All of these cases histopathologically showed typical intravascular localization of the neoplastic cells in the initial biopsy specimens obtained from sites other than the CNS. Despite multiagent chemotherapy, patients suffered from single or multiple CNS mass lesions 5-44 months after the initial diagnosis of IVL, except for one case in which IVL and the CNS mass lesion were diagnosed at the same time. The subsequent biopsy and autopsy specimens obtained from the CNS mass lesions revealed diffuse infiltration of the tumor cells with perivascular spreading, but minimal or no intravascular components. Immunohistochemical analysis of intravascular tumor cells and CNS mass lesions revealed expression of CD20, CD79a, bcl-2 and negative for CD3e and Epstein-Barr virus encoded RNA. The overall features of the CNS mass lesions were very similar to or indistinguishable from those of the primary CNS lymphomas. This implies that CNS mass lesions in the IVL cases can be correctly diagnosed only by careful attention to clinical and pathological findings. Moreover, there is the possibility that some cases previously diagnosed as primary CNS lymphomas may have include IVL cases. Further investigation is needed to explore this unusual phenomenon.     

Morphologie neurologischer Komplikationen bei der intravaskulären Lymphomatose

Intravascular lymphomatosis (IVL) is a rare lymphoproliferative disease characterized by intravascular growth of lymphoma cells in small vessels. Most frequently, its first manifestations occur in the central nervous system or skin. Based on autoptical findings in a 68-year-old-man with IVL, the pathological morphology in the central nervous system is compared to the course of neurological symptoms. The disease could not be diagnosed during his lifetime. The spectrum of neurological deficits can be explained histopathologically by occlusive intravascular aggregations of lymphomatous cells in small vessels of the spinal leptomeninges and nerve roots causing perfusion deficits and areas of ischemic necrosis of up to 4 mm in diameter in the medulla. Aggregations of lymphoma cells in the vessels of other organs are also found, but did not result in clinical symptoms. In reference to the present case, a survey of other literature reports on IVL with neurological and psychiatric manifestations is given. In patients with this condition, even an invasive biopsy has to be considered in order to make the correct diagnosis at an early clinical stage.     

Intravascular lymphomatosis of the lung and liver following eyelid lymphoma in a Chinese man and review of primary pulmonary intravascular lymphomatosis

Intravascular lymphomatosis (IVL) is characterised by an almost exclusive intravascular proliferation of malignant lymphoid cells, with the diagnosis often made only when the illness is in its terminal phase or at autopsy. We detail a case of IVL affecting the lung and liver of a 49-year-old Chinese man presenting primarily with lung symptoms and incidental findings of abnormal serum transaminase levels, the ante-mortem diagnosis being established on transbronchial lung biopsy and percutaneous liver biopsy specimens, respectively. Histology disclosed CD20 + CD5 - CD10 [corrected] - malignant large mononuclear B cells within the lumina of the blood vessels of the affected organs as well as sinusoids of the liver. Significantly, the patient had a history of large B cell lymphoma affecting the eyelid 18 months prior to the angiotropic disease, suggesting a possible link between the more common types of non-Hodgkin's lymphoma and IVL. A brief review of all cases of primary pulmonary intravascular lymphomatosis is also presented.     

Cutaneous intravascular natural killer/T cell lymphoma with peculiar immunophenotype

Intravascular lymphoma (IVL) is a rare entity. Most cases are a variant of extranodal diffuse large B cell lymphoma, and fewer than 10% of the published cases are of T cell origin. Only intravascular B cell lymphoma is recognized as a distinct entity in the most recent World Health Organization (WHO) classification of lymphoproliferative disorders. We describe a case of cutaneous natural killer (NK)/T IVL, with a cytotoxic immunophenotype and Epstein–Barr virus (EBV) positivity. However, our case was immunohistochemically negative not only for T cell receptor (TCR)‐βF1 and TCR‐γ (TCR‐silent), but also for CD56, making it the first triple‐negative NK/T IVL case to be described. We urge recognition of this NK/T cell lineage intravascular lymphoma due to its particular immunophenotypical profile and its unvarying relationship with EBV. Its occurrence should not be considered a coincidence, but rather a key aspect of the pathogenic background of this haematological neoplasm.     

Large-cell anaplastic (Ki-1-positive) lymphoma complicated by disseminated intravascular coagulation.

Large-cell anaplastic lymphoma (Ki-1-positive lymphoma) was first described as a type of large-cell lymphoma that has morphologic, enzymatic, and immunologic similarities to malignant histiocytosis. To date, an association between Ki-1-positive lymphoma and disseminated intravascular coagulation has not been described, but cases of malignant histiocytosis with disseminated intravascular coagulation have been reported. We report a case of anaplastic, Ki-1-positive, large-cell lymphoma complicated by clinical and laboratory evidence of disseminated intravascular coagulation with histologic evidence of vascular invasion and fibrin thrombi.     

Association between necropsy evidence of disseminated intravascular coagulation and coagulation variables before death in patients in intensive care units.

The necropsy findings in 21 patients on an intensive care unit, on whom coagulation studies had been performed immediately before death, were assessed. Eleven of the patients were retrospectively studied and 10 were reviewed consecutively in a prospective study. Fifteen patients (eight retrospective and seven prospective) had evidence of disseminated intravascular coagulation. Microthrombi were most often found in the lungs and kidneys. The most common abnormal coagulation tests in patients with necropsy evidence of disseminated intravascular coagulation were raised serum concentrations of fibrinogen and fibrin degradation products, prolonged prothrombin time, and reduced platelet counts. Reduced fibrinogen concentrations and a prolonged thrombin time were the least commonly observed abnormalities. There was no difference in either the prevalence or magnitude of abnormality of any particular coagulation variable test result between those patients with evidence of disseminated intravascular coagulation at necropsy and those without.     

Neisseria meningitidis serogroup 29E (Z'') septicemia in a patient with far advanced multiple myeloma (plasma cell leukemia).

A case of septicemia caused by Neisseria meningitidis serogroup 29E (Z') in a patient with plasma cell leukemia is described. The patient developed disseminated intravascular coagulation, had a cardiopulmonary arrest, and died. The effects of altered immune function leading to a predisposition to meningococcal infections are discussed.     

The pathological anatomy of pneumococcal meningoencephalitis in adults

The results of the clinico-morphological examination of the bacteriologically confirmed meningoencephalitis in 12 autopsy cases are presented. The gravity of the clinical course of the pneumococcus meningoencephalitis is due to infectious-toxic shock the main morphological manifestation of which is disseminated intravascular coagulation. Neurotoxicosis symptoms prevailed in the clinical picture of the infectious-toxic shock even though the morphological changes characteristic of shock were found in the brain as well as in the internal organs particularly in the adrenals, lungs and kidneys. The peculiar feature of the disseminated intravascular coagulation was the domination of the hypercoagulation stage with the variety of intravascular fibrin formations (fibrin, hyaline, globular thrombi, prethrombi, fibrinous threads).     

Disseminated intravascular coagulation in the practice of the pathologist]

Disseminated intravascular coagulation (DIC) of blood develops as a result of a sharp increase in the release of thromboplastic substances. The mechanism of disseminated thrombosis is switched in at the level of the microcirculatory bed with defibrination of the peripheral blood and subsequent hemorrhages and bleedings. The causes of DIC development may include complications of pregnancy and delivery, different kinds of shock including endotoxin shock, hemorrhage, hemolysis. Histomorphological findings in DIC are as follows: hemorrhagic syndrome, fibrin thrombi in capillaries, arterioles and venules of the skin, kidneys, adrenals, hypophysis, gastrointestinal tract, lungs and other organs followed by necroses and hemorrhages in these organs. Clinically, DIC is manifested by symptoms of insufficiency of the affected organs (acute renal insufficiency, Waterhouse-Fridericksen syndrome, etc).     

The pathology of experimental Ebola virus infection in monkeys.

Six rhesus and two vervet monkeys were infected intraperitoneally with Ebola virus. They developed an acute haemorrhagic fever with skin rash 4 days later and died 6--12 days after infection. Histopathological lesions of acute necrosis were present in the liver, spleen, lymph nodes, lungs and testes. The presence of fibrin thrombi in several organs was suggestive of the occurrence of disseminated intravascular coagulation during the infection.     

Pancreatic ischaemic lesions without fat necrosis associated with disseminated intravascular coagulation

We examined the primary ischaemic changes in the pancreas in 35 patients with disseminated intravascular coagulation. In 7 (20%), multiple patchy lesions composed of degenerative acinar cells indicating coagulation necrosis were noted. None of these lesions was accompanied by fat necrosis. The patchy lesions involved the islets of Langerhans in only 1 case. The interlobular arteries of the pancreas near these lesions contained fibrin thrombi in all 7 cases. We suggest that these lesions, without fat necrosis, are the distinctive ischaemic change associated with disseminated intravascular coagulation.     

<Emphasis Type="Italic">Plasmodium falciparum</Emphasis> Cerebral Malaria Complicated by Disseminated Intravascular Coagulation and Symmetrical Peripheral Gangrene: Case Report and Review

The case of a 56-year-old female tourist who survived cerebral Plasmodium falciparum malaria with disseminated intravascular coagulation and symmetrical peripheral gangrene, ultimately requiring amputation of her left-sided fingertips and toes, is reported. While symmetrical peripheral gangrene has been described rarely in Asian, African, and American patients with Plasmodium falciparum malaria and disseminated intravascular coagulation, no such case has been reported in travelers returning from endemic areas.     

Nodal CD8 positive cytotoxic T-cell lymphoma: a distinct clinicopathological entity.

We studied 11 cases of nodal cytotoxic T-cell lymphoma, which express the CD8+ phenotype and cytotoxic molecules (T-cell intracellular antigen-1, granzyme B and perforin), to characterize the clinicopathologic spectrum of these neoplasms. The 11 cases consisted of four men and seven women, aged 5 to 82 years (mean, 53 years). All cases were nodal, and eight of 11 had extranodal involvement, the most common being in bone marrow (eight cases) and liver (six cases). The expression of these cytotoxic molecules has been reported in some T/natural killer cell lymphomas mostly involved in extranodal sites of skin, nasopharyngeal region, or gastrointestinal tracts, but these types of extranodal involvement were rare in our cases. Morphologically these lymphomas could be divided into two groups. One group (n = 6) showed a diffuse large cell type and massive necrosis or apoptosis that was accompanied by disseminated intravascular coagulation (DIC) or hemophagocytic syndrome (HPS) on the initial. The prognosis of this group was generally poor (survival = 1-19 months, median = 5 mo), and four of these six cases were fulminant. The other group (n = 5) showed a diffuse medium or mixed cell type, and the prognosis was not so poor (median survival = 17 mo). Our results suggest that these nodal cytotoxic T-cell lymphomas originated from activated cytotoxic T-cells and were highly accompanied with DIC or HPS.     

A Fatal Case of Intravascular Coagulation After Bee Sting Acupuncture

Bee stings can cause severe adverse reactions, leading to anaphylaxis, cardiovascular collapse, and death. In some cases, bee venom also induces disseminated intravascular coagulation (DIC). However, to our knowledge, there has been no fatal case of intravascular coagulation accompanied by anaphylaxis caused by bee sting acupuncture. Here, we report a fatal case of a 65-year-old woman with DIC, following anaphylactic shock after bee sting acupuncture. This case emphasizes that practitioners should consider anaphylaxis followed by coagulation abnormalities when a patient''s vital signs are unstable after bee sting acupuncture.     

Disseminated intravascular coagulation induced by generalized cytomegalic inclusion disease during steroid therapy for polymyositis

A 56-year-old woman came to the hospital with fever and skin eruptions. A rise in myogenic enzyme and the presence of antileucocyte antibody were noticed, along with the gradual appearance of myalgia in both lower extremities, and muscle weakness. Steroid therapy was started under the diagnosis of polymyositis. The steroid was reduced because of mental disturbance but immediately the patient developed high fever. Various forms of treatment were carried out but there was no improvement, and the patient died. At autopsy there were scattered purpura on the skin, and the muscles were atrophic and yellowish-grey in color. Histopathologically, there was inflammatory cell infiltration and muscle fiber degeneration visible in many of the muscles, and the findings showed evidence of polymyositis. There were intranuclear inclusions in the lungs, ovaries, and adrenal glands, and this was diagnosed as generalized cytomegalic inclusion disease. Fibrin thrombi were found in the kidneys, lungs, and adrenal glands and this was pathologically diagnosed as disseminated intravascular coagulation. Endothelial cell damage caused by cytomegalovirus was assumed to be involved to a large extent in triggering the disseminated intravascular coagulation.     

DISSEMINATED INTRAVASCULAR COAGULATION INDUCED BY GENERALIZED CYTOMEGALIC INCLUSION DISEASE DURING STEROID THERAPY FOR POLYMYOSITIS

A 56-year-old woman came to the hospital with fever and skin eruptions. A rise in myogenic enzyme and the presence of antileucocyte antibody were noticed, along with the gradual appearance of myalgia in both lower extremities, and muscle weakness. Steroid therapy was started under the diagnosis of polymyositis. The steroid was reduced because of mental disturbance but immediately the patient developed high fever. Various forms of treatment were carried out but there was no improvement, and the patient died. At autopsy there were scattered purpura on the skin, and the muscles were atrophic and yellowish-grey in color. Histopathologically, there was inflammatory cell infiltration and muscle fiber degeneration visible in many of the muscles, and the findings showed evidence of polymyositis. There were intranuclear inclusions in the lungs, ovaries, and adrenal glands, and this was diagnosed as generalized cytomegalic inclusion disease. Fibrin thrombi were found in the kidneys, lungs, and adrenal glands and this was pathologically diagnosed as disseminated intravascular coagulation. Endothelial cell damage caused by cytomegalovirus was assumed to be involved to a large extent in triggering the disseminated intravascular coagulation. ACTA PATHOL. JPN. 35: 723–730, 1985.