ALK+ Anaplastic large cell lymphoma with extensive cardiac involvement: A rare case report and review of the literature

Cardiac lymphoma is a rare entity. In this setting, the secondary involvement of the heart is far more frequent than the primary cardiac lymphoma. Herein, we present an autopsy case of a disseminated anaplastic lymphoma kinase (ALK)-positive anaplastic large cell lymphoma with a dominant mediastinal involvement. Extensive cardiac infiltration with the near replacement of the myocardial wall by the neoplastic cells was observed. A total of nine isolated case reports of anaplastic large cell lymphoma with cardiac involvement were found in the English-language literature, and a widespread cardiac and thymic infiltration by the systemic ALK-positive anaplastic large cell lymphoma has not been documented. An incidental regenerative nodule was also identified in the liver. The patient died of pulmonary thromboembolism and cardiac arrest.

Liver pathology in collagen vascular disorders highlighting the vascular changes within portal tracts.

Background: Collagen vascular disorders (CVDs) are autoimmune disorders with multisystem involvement. Clinical liver involvement is not a characteristic feature though histological involvement could be frequent. Liver disease in CVDs could be the consequence of various factors. Aim: The aim was to analyze the histological spectrum of liver in collagen vascular disorders (CVDs) at autopsy. Materials and Methods: Thirty-six autopsy livers negative for hepatitis B or C virus were studied in CVD cases with no known association with chronic liver disease or vascular thrombosis or hematological disorder. Cirrhotic and normal livers were used as controls. The paired t-test, one-way ANOVA, and two-sided Dunnett t-test were used for comparison (< 0.05). None of the control cases showed any abnormal vessels. Results: There were 21 systemic lupus erythematosus (SLE), 7 rheumatoid arthritis (RA), 5 systemic sclerosis (SSc), and 3 polyarteritis nodosa (PAN) cases (M:F = 11:25, age range 23-60 years). Histology: Diffuse nodular regenerative hyperplasia of liver (NRHL) was seen in 10 cases, and 6 (5 SLE and 1 RA) had numerous abnormal thin-walled vessels in intermediate- and small-sized portal tracts with no vascular occlusion or inflammation. Moderate sized portal tracts showed more interface and lobular inflammation. The main portal vein and its major branches were normal. None of these six cases had increased transmainases (P>0.05). Most SLE cases had increased transaminases (P<0.05). No evidence of portal hypertension was seen in all except in one RA. Septicemia is known to be associated with raised transaminases. Conclusion: A rare pathology of conglomerate of abnormal vessels in intermediate- and small-sized portal system was observed co-existing with NRHL in CVDs. Raised liver enzyme with interface hepatitis in CVD may not necessarily warrant an overlap, as a similar feature could be observed in septicemia.

Modification of small bowel microflora in chronic alcoholics with alcoholic liver disease.

The bacterial flora of the gastrointestinal tract plays an essential role in human physiology. Our aim was to study the pattern of change in bacterial microflora in the small intestines of patients with chronic alcoholic liver disease (ALD). Patients of ALD made up the test group and nonalcoholics served as controls. Duodenal (D2) biopsies were obtained by upper gastrointestinal endoscopy and processed immediately for microbiological analysis. Marked qualitative and quantitative alterations of small intestinal microflora was documented in chronic alcoholics. There was increased bacterial growth of both gram-positive cocci and gram-negative bacilli in the ALD group.

Comparison of multidetector computed tomographic colonography and conventional colonoscopy for detection of colorectal polyps and cancer.

BACKGROUND: Computed tomographic colonography (CTC) is a new technique for detecting colonic neoplasms. Data on the utility of this method in the Indian population are limited. METHODS: Forty-two patients with symptoms of colonic disease underwent CTC and conventional colonoscopy (CC) within one week of each other and the findings at these two investigations were compared. RESULTS: The entire colon could be evaluated in 38 patients on CTC and in 23 patients on CC. Of the 19 patients who had incomplete CC, 14 had occlusive colonic lesions. Of the 86 lesions detected on CC, 76 (88.4%) were correctly identified on CTC with regard to location and size. CTC was false negative for 10 lesions and false positive for 5 lesions in 3 patients. The sensitivity and specificity of CTC were 65% and 77%, respectively, for lesions 1-5 mm; 97% and 83% for 6-9 mm-sized lesions; and 100% and 100% for lesions 10 mm or larger. Extracolonic findings were seen in 24 of 42 patients (57%). CONCLUSIONS : CTC is reliable for detecting lesions 6 mm or larger in size. It permits evaluation of the region proximal to an occlusive growth, which is often not possible with CC.

Multiple spontaneous enterocutaneous fistulae in malakoplakia.

Malakoplakia is a rare chronic inflammatory disease, usually involving the urogenital tract. We report a patient who presented with a psoas abscess, and later developed colocutaneous fistula at the site of abscess drainage and multiple spontaneous fistulae away from the site of incision. Histology of the resected specimen showed Michaelis-Gutmann bodies, which are diagnostic of malakoplakia.

Pulmonary and hematological alterations in idiopathic ulcerative colitis.

BACKGROUND: Patients with idiopathic ulcerative colitis (IUC) may suffer from one or more extraintestinal manifestations. We decided to prospectively study the prevalence of extraintestinal manifestations among patients with IUC, with special reference to pulmonary and hematological alterations. METHODS: Fifty-one consecutive patients with IUC attending the gastroenterology services of our tertiary-care referral center were evaluated prospectively. A detailed clinical evaluation of the musculoskeletal system, eye and skin, X-ray examination of the sacroiliac joints and chest, liver function tests, coagulation profile, hemogram, pulmonary function tests (PFT) and diffusion capacity for carbon monoxide (DLCO) were done in all patients. ERCP, liver biopsy and high-resolution computerized tomography (HRCT) of the chest were performed whenever indicated. RESULTS: Nearly half (24/51; 47%) the patients had one or more extraintestinal manifestations; these included pulmonary function abnormalities (14 patients; 27%), sacroiliitis (8; 16%), arthritis (5; 10%), ocular complications (4; 8%), and pyoderma gangrenosum and Budd-Chiari syndrome (one patient each). Of the 14 (27%) patients who had abnormal pulmonary function, isolated PFT abnormalities were seen in 8 (restrictive pattern in 7, obstructive pattern in 1), decreased DLCO in four, and both of the above in two. All patients with decreased DLCO (n=6) were asymptomatic and had normal chest X-ray and HRCT chest. Thirteen (25%) patients had thrombocytosis, 19 (37%) had increased fibrinogen level, and one patient had decreased antithrombin level. Disease activity had significant association with decreased DLCO (p=0.008), increased platelet count (p<0.0001), increased fibrinogen level (p=0.016), low antithrombin levels (p=0.046) and arthritis (p=0.002). CONCLUSION: Extraintestinal manifestations of IUC were seen in 47% of patients. Asymptomatic pulmonary interstitial involvement was seen in 12%, more often among patients with active disease.

True epiphrenic diverticulum of esophagus secondary to tubercular adenitis.

Ephiphrenic esophageal diverticulum is rare and usually is a pulsion-type pseudodiverticulum that occurs in association with motility disorders of the esophagus. We report a 35-year-old man with tubercular lymphadenitis causing true traction type of epiphrenic diverticulum. Esophageal diverticulectomy and esophagomyotomy was done; he is doing well on antitubercular drugs.