Malignant histiocytosis: a case report of a rare tumour presenting with spontaneous splenic rupture

Malignant histiocytosis is a rare invasive proliferation of neoplastic histiocytes. Cases previously reported as malignant histiocytosis were shown to be lymphomas of T or B lineage, especially anaplastic large-cell lymphomas. A case of malignant histiocytosis is described, in which a patient presenting with symptoms suggestive of pneumonia suddenly deteriorated and died. At autopsy, a large quantity of fresh blood, originating from several ruptured nodules on the enlarged spleen, was seen in the peritoneal cavity. Extensive infiltration by pleomorphic tumour cells and erythrophagocytosis by tumour cells were seen on histological examination of the spleen. Immunohistochemical analysis and staining were carried out. This is the second reported case of malignant histiocytosis presenting with spontaneous splenic rupture.     

Systemic granulomatous necrotizing vasculitis in a MPO-ANCA-positive patient

We present a case of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-associated vasculitis that demonstrated a systemic granulomatous lesion at autopsy. The patient initially showed anorexia, general malaise and anemia. Colon fiber was examined to detect the bleeding site, which revealed ischemic mucosal damage associated with venous fibrin thrombus. Because a high titer of MPO-ANCA was found, ANCA-associated vasculitis was suspected and the patient was started on steroid pulse therapy. However, anemia, renal failure and respiratory failure worsened and the patient died of sudden cardiac failure 2 days after the start of the therapy. An autopsy revealed systemic arteritis in multiple organs including the kidneys, liver, spleen, gastrointestinal system and genital organs that indicated fibrinoid necrosis accompanied by granulomatous reaction with multinucleated giant cells; the granulomatous reaction further extended along the splenic capsule. Glomerulonephritis and diffuse pulmonary damage, which are common in MPO-ANCA-associated vasculitis, were almost absent but parapleural fibrosis was present. The direct cause of death was presumed to be hemorrhagic shock due to rupture of an aneurysm in the gastric subserosa. As far as we know, this is the first case of a systemic granulomatous reaction in MPO-ANCA-positive vasculitis, although the cause of the granulomatous lesion is unknown.     

Subvalvular left ventricular false aneurysm complicating mitral valve replacement

Surgical replacement of the mitral valve by a prosthetic valve may lead to localized disruption of atrioventricular continuity resulting from direct surgical trauma or from delayed rupture of the weakened annulus. Such disruption leads to subepicardial hematoma formation that may remain localized if pericardial adhesions are present. Of two patients who suffered this complication, the first died of rupture of the epicardial hematoma. The hemorrhage in the second patient was contained by pericardial adhesions and had organized, thus resulting in a subannular left ventricular false aneurysm. The aneurysm was found incidentally at autopsy after the patient died of cerebral embolism from prosthetic valve thrombi.     

A case of Penicillium marneffei infection in an AIDS patient: the first case in Japan.

A 38-year-old Japanese AIDS patient developed papular lesions which rapidly increased in number, eroded and crusted, and spread over not only skin but also the mucosal surface. High fever, sore throat, malaise and hepatosplenomegaly were also noted, and he died despite 2 months of intensive treatment. An autopsy revealed numerous histiocytes infected with Penicillium marneffei in the lymph nodes, liver, spleen, bone marrow, skin, and mucosal surface of the oral cavity to the pharynx. This case is thought to be the first Japanese case of penicilliosis marneffei.     

Visceral botryomycosis in a case of Erysipelothrix rhusiopathiae endocarditis

Botryomycosis is a rare bacterial infection of the skin and, rarely, viscera that is characterized by the formation of characteristic hyaline grains. We encountered a patient with Erysipelothrix rhusiopathiae endocarditis who developed visceral botryomycosis. He was a 54-year-old black man who presented in sepsis with a history of progressive weakness and severe weight loss. He died 10 hours after admission. At autopsy, endocarditis was identified, along with infarcts of the spleen and kidneys. Microscopically, visceral botryomycosis was seen. With these bacteria, an animal source is usually identified, although one was not found in this man. Erysipelothrix rhusiopathiae is an organism that is becoming identified in a range of infections in humans, the manifestations and pathophysiology of which are still being discovered.     

Acquired immunodeficiency syndrome associated with Acanthamoeba infection and other opportunistic organisms

A 29-year-old Haitian man with acquired immunodeficiency syndrome presented with nasal obstruction and epistaxis. A computed tomogram of the head showed thickened nasal and paranasal sinus mucosa. A biopsy specimen of the turbinate disclosed inflammatory tissue containing amoebic trophozoites. The patient was empirically treated with rifampin and ketoconazole. He died four months after biopsy of other complications of acquired immunodeficiency syndrome. At autopsy, the amoebic infection was found only in the paranasal sinuses, a calf nodule, and in an intradermal abscess in the left leg. Pneumocystitis carinii pneumonia, Mycobacterium avium-cellulare in the liver and retroperitoneal lymph nodes, cytomegalovirus infection of the adrenal glands, and Kaposi's sarcoma in the spleen were additionally present. The organism was cultured and studied by electron microscopy, dark-field microscopy, and immunofluorescence.     

A case of Wegener's granulomatosis with involvement of the aortic valve

A clinico-morphological observation of Wegener granulomatosis in a 29-year-old man is described. The disease started with the gastrointestinal disorders followed by lung and renal disturbances which dominated. Heart enlargement and systolic-diastolic noise in the Botkin point were found. Infectious endocarditis with a complex aortal defect were diagnosed. The patient died 6 months later. The autopsy revealed a necrotizing lung granulomatosis, disseminated vasculitis, renal and spleen infarcts. Vasculitis with granulomatosis, destructive changes in the intima were found in the supravalvular part of the aorta and at the base of the aortic valve as well as severe fibrinous valvulitis.     

Ischemic stroke and splenic rupture in a case of Streptococcus bovis endocarditis

A 58-year-old man with an acute stroke suffered from splenic rupture. Streptococcus bovis was found in blood cultures, and gram-negative cocci were found in the infarcted spleen. Hemorrhagic transformation of the stroke occurred. Echocardiography showed aortic endocarditis. Cardiac surgery was not performed because of concern about cerebral bleeding. The patient died due to cerebral rehemorrhage after 3 weeks.     

Primary splenic lymphoma with filiform ultrastructure.

A case of primary large cell splenic lymphoma of B lineage exhibiting filiform cell appearance is reported. The patient presented with massive splenomegaly, and following spontaneous splenic rupture, died of adult respiratory distress syndrome. The clinical aspects of the case, notably a lymphoma arising as a primary tumour in the spleen, with spontaneous spleen rupture and rapid fatal outcome, in combination with the filiform appearance of the lymphoma on electron microscopic examination, constitute an unusual combination of features. As far as is known, this B cell neoplasm is only the second primary splenic lymphoma of filiform type to be recorded.     

CANCER OF GALLBLADDER WITH SEVERE THROMBOCYTOSIS

An autopsy case of severe thrombocytosis associated with cancer of gallbladder is described as the erst case in the literature. A 78-year-old male patient was admitted to the hospital with abnormal hepatic functions on August 6,1975. Laboratory examination revealed platelet count of 5,664,0001 cu mm, anemia, leukocytosis and bilirubinemia. Jaundice was progressive and the patient died after a month. Severe thrombocytosis persisted throughout the course. Main autopsy findings were cancer of gallbladder with metastasis to the liver, fibrosis of spleen and purulent bronchopneumonia. A possible mechanism of unusually severe degree of thrombocytosis associated with the present case is discussed in light of the literature.     

The autopsy in acquired immunodeficiency syndrome. How and why

The autopsy on the patient who has died of acquired immunodeficiency syndrome (AIDS) can be of great value to the physicians who cared for the patient and to society in general. Although the AIDS virus has been shown to be significantly contagious in the clinical setting, there is no evidence thus far that it is particularly contagious in the autopsy room. Indeed, there is no documentation of a pathologist or autopsy room assistant having contracted AIDS or having seroconverted because of the performance of an autopsy on a patient with AIDS. Procedures to follow in the autopsy of the patient with AIDS are described. The hospital pathologist can safely examine the patient who has died of AIDS by using the well-established techniques for performance of the autopsy.     

Spontaneous rupture of the spleen associated with pneumonia

Spontaneous rupture of the spleen is a rare and life-threatening complication of bacterial pneumonia, only six properly documented cases having been reported to date. A case of spontaneous splenic rupture associated with pneumonia caused byLegionella pneumophila is presented, together with a review of the literature. Most of the patients were aged over 50, but none had predisposing conditions. Left lung involvement predominated. Legionellosis and Q fever were the most frequent etiologic diagnoses. Empiric antibiotic therapy was adequate in all but two patients. One patient died; he had not undergone laparotomy. Spontaneous rupture of the spleen is an extremely rare complication of bacterial pneumonia that endangers the patient's life if surgery is not performed immediately. This complication should be borne in mind in patients with atypical pneumonia who have left quadrant pain and a falling hematocrit, even in the absence of prior splenomegaly.     

Cytomegalic disease as a cause of disseminated lung lesions in an immunosuppressed patient

A case of disseminated lesions in the lungs was diagnosed just during autopsy as cytomegalic infection. 54-year old patient treated since 10 years because of lymphoplasmacytoid lymphoma of low malignancy was admitted to Institute of Tuberculosis with suspicion of miliary tuberculosis. The high temperature, pemphigus--like skin lesions and disseminated lesions in the chest X-ray appeared immediately after succeeding chemotherapy. Tuberculous bacilli. Aspergillus fumigatus and Pneumocystis carinii were not found in examined materials (BALF, blood, urine, skin and mucous lesions). Patient died after 3 days and typical lesions of cytomegalovirus infection were found only after autopsy in macroscopic picture of the lungs.     

Angiotropic lymphoma (intravascular lymphomatosis)--2 case reports

Described are two cases of angiotropic lymphoma where eventually autopsy elucidated nonspecific neurologic symptoms. One patient suffering ambiguous encephalitic syndrome died three months later, the second one passed away after an unusually long three-year period of progressive dementia and cumulative motoric dysfunction. The autopsy disclosed pure intravascular malignant lymphoid aggregates (LCA, CD 20, Bcl 2-positive) in the brain and kidney of both patients. In the patients with the longer disease period, a dissemination to lung was also found. Definitive diagnosis was issued as a B cell type of angiotropic lymphoma. Skin, lymph nodes, spleen, and bone marrow were not affected in any case. The clinical differential diagnosis algorithm did not involve this rare etiology in these particular uncommon neurologic cases and even brain biopsy performed in both women did not recognize the substantiality of the disease.     

HEREDITARY HEMORRHAGIC TELANGIECTASIA WITH MALIGNANT LYMPHOMA An Autopsy Case

A 60-year-old Japanese woman was diagnosed at autopsy as having had hereditary hemorrhagic telangiectasia (HHT) associated with systemic hemangiomas. In her repoduction period, premenstrual epistaxis frequently occurred. At the age of 60, the patient died of malignant lymphoma. At autopsy, multiple telangiectatic spots were noted on the face, limbs and trunk. The paraaortic lymph nodes, which were enlarged and irregularly conglomerated, were histologically diagnosed as malignant lymphoma of the diffuse large cell type. Submucosal telangiectatic lesions were found in the gastrointestinal system from the oral cavity to the rectum. Cavernous hemangiomas were present in various visceral organs including the liver, spleen, small and large intestines, rectum, appendix, uterus, and jejunal and colonic mesenteries. There was an arteriovenous fistula in the left lung. Examination of her family pedigree showed that the patient had an autosomal dominant trait of inheritance. The pathogenesis of the systemic visceral hemangiomas observed in this patient was considered to be similar to that of hamartoma.     

Hereditary hemorrhagic telangiectasia with malignant lymphoma. An autopsy case

A 60-year-old Japanese woman was diagnosed at autopsy as having had hereditary hemorrhagic telangiectasia (HHT) associated with systemic hemangiomas. In her reproduction period, premenstrual epistaxis frequently occurred. At the age of 60, the patient died of malignant lymphoma. At autopsy, multiple telangiectatic spots were noted on the face, limbs and trunk. The paraaortic lymph nodes, which were enlarged and irregularly conglomerated, were histologically diagnosed as malignant lymphoma of the diffuse large cell type. Submucosal telangiectatic lesions were found in the gastrointestinal system from the oral cavity to the rectum. Cavernous hemangiomas were present in various visceral organs including the liver, spleen, small and large intestines, rectum, appendix, uterus, and jejunal and colonic mesenteries. There was an arteriovenous fistula in the left lung. Examination of her family pedigree showed that the patient had an autosomal dominant trait of inheritance. The pathogenesis of the systemic visceral hemangiomas observed in this patient was considered to be similar to that of harmartoma.     

An immunofluorescent study of generalized Coxsackie virus B3 infection in a newborn infant

An autopsy case of a 10-day-old newborn with generalized infection of Coxsackie virus B3 (CBV3) was reported. CBV3 was isolated from the blood before death. The patient died of cardiac failure. An immunofluorescent study was carried out on autopsy specimens fixed in formalin and embedded in paraffin. CBV3 antigen was detected in the heart, brain, kidney, lungs, spleen, thymus, and pancreas. In the pancreas CBV3 antigen was predominantly seen in the islet cells. No CBV3 antigen was found in the liver and adrenal glands. Electron microscopic examination revealed virion-like particles, 20 nm in diameter, in the endothelial cells of the myocardium.     

Myelomalacia caused by embolism of the substantia gelatinosa in the vertebral column and spinal cord arteries

A 56 year old woman developed a transverse lumbar paraplegia within two hours following a jump from an 80 centimeter high window. The patient died 13 days later from a pulmonary embolus. At autopsy an intervertebral disc tissue embolus was found in the arteries to the 12th thoracic vertebral body and the spinal cord. The embolus originated in a Schmorl nodule in the 12th thoracic vertebral body. The findings and the clinical course suggest that dislodged nucleus pulposus tissue was expressed into the vascular system over some time as a result of tears in small vessels. The implications for treatment of patients with similar spinal cord symptoms are considered.     

Mast cell leukemia with rapidly progressing portal hypertension

Reported herein is an autopsy case of mast cell leukemia, a rare form of systemic mastocytosis, complicated with portal hypertension. A 52-year-old woman presented with urticaria-like skin symptoms, anemia, and thrombocytopenia. Atypical mast cells (CD2+, CD25+, CD117+) with toluidine blue metachromasia were found in the peripheral blood and on bone marrow aspiration smears. Chemotherapy with cytosine arabinoside and idarubicin was ineffective and the patient died of multi-organ failure with rapidly progressing hepatosplenomegaly and large-volume ascites 3 months after admission. At autopsy the bone marrow, spleen, liver, and lymph nodes were extensively infiltrated by atypical tumor cells with occasional bi- or multi-lobated nuclei. They were positive for mast cell tryptase and possessed an activating mutation of the c-kit gene (D816V). Ascites (2200 mL) and non-ruptured esophageal varices with submucosal hemorrhage indicated the presence of severe portal hypertension. Although there was no evidence of liver cirrhosis, the hepatic sinusoids were clogged with tumor cells, with a tendency to be more severe in the perivenular areas, and the lumens of central veins were obliterated by tumor cell infiltration. The present case demonstrates that non-cirrhotic portal hypertension due to blocking of sinusoidal and venous flow could be a serious complication in mast cell leukemia.     

HISTIOCYTIC MEDULLARY RETICULOSIS

An autopsy case of histiocytic medullary reticulosis (HMR) in a 55-year-old man is presented. The initial complaint of the patient was an unusual skin lesion on the right upper arm, and subsequently developed generalized erythematous skin lesions which regressed during the course of the illness. The clinical symptoms and course of the disease were those of HMR. The affected organs were lymph nodes, spleen, liver and bone marrow as well as the skin, in which erythrophagocytic histiocytes predominated in abnormal cells of the histiocytic series. Phagocytic histiocytes had large amounts of cholesterol and small amounts of phospholipids which might be originated from the ingested erythrocytes. The electron microscopy suggested the presence of phospholipid-cholesterol interaction in the phagocytic histiocytes.