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Histiocytic medullary reticulosis with parallel increases in serum acid phosphatase and disease activity.

Two months after presenting with autoimmune hemolytic anemia, a 57-year-old man developed increased serum acid phosphatase activity. Subsequently, abnormal histiocytes were found in the bone marrow and blood, and 8 months after the onset of his illness lymph node biopsy led to the diagnosis of histiocytic medullary reticulosis. At that time the level of serum acid phosphatase activity was more than 60 times the upper limit of normal and then paralleled the activity of his disease during temporary responses to chemotherapy, rising to over 90 times normal at the time of his death 1 year after presentation. No prostatic malignancy was demonstrated by biopsies or at autopsy, and electrophoretic studies of the serum enzyme suggested that it was of extraprostatic origin. It is postulated that the acid phosphate present in the serum at levels heretofore described only in metastatic carcinoma of the prostate may have arisen from the abnormal histiocytes.

Acid Phosphatase↗

Histopathology of the conducting tissue of the heart in Chagas' myocarditis.

The conducting tissue of the heart was studied in 25 human cases of Chagas' myocarditis with a method which employs complete serial sections mounted on continuous transparent plastic tape. The pathological changes were correlated with electrocardiographic findings. The inflammation of the acute phase of Chagas' myocarditis, as seen in one single case, did not seem to interfere with conduction through the AV system. In chronic Chagas' myocarditis the conducting tissue showed extensive and variable changes: chronic inflammation, fibrosis, atrophy and fragmentation of specific fibers, extreme dilatation and tortuosity of veins, capillaries and lymphatics, fatty infiltration, and arterial medial and intimal fibrosis. A preferential involvement of the right bundle branch and the anterior fascicles of the left branch was observed and an excellent correlation with electrocardiographic abnormalities was found. There was also evidence presented that bundle branch block may be caused by disease proximal to the bundle branches. Complete AV block seemed to be the final result of the progressive inflammatory and degenerative changes involving the conduction system in chronic Chagas' myocarditis. Inflammation and fibrosis did also involve the sinoatrial node, Purkinje fibers, intracardiac nervous ganglia, and the contractile myocardium.

Acute Disease↗

Benign glandular inclusions in lymph nodes, endosalpingiosis, and salpingitis isthmica nodosa in a young girl with clear cell adenocarcinoma of the cervix.

A 12-year-old girl underwent radical surgery for clear cell adenocarcinoma of the endocervix. Bilaterally dilated fallopian tubes with hyperplastic mucosal folds and salpingitis isthmica nodosa were found in association with benign glandular inclusions in a para-aortic lymph node and in the serosa of pelvic organs. Changes in the müllerian-derived epithelium appear to be a common denominator of these unusual lesions in this young girl. A primary growth disturbance of the cells of the müllerian system, which was conditioned during embryonic development and manifested itself later in life in response to appropriate stimuli associated with menarche, is postulated as the underlying abnormality.

Adenocarcinoma↗

Pulmonary histology for the surgical pathologist.

This is a review of the variety of pulmonary histologic artifacts and incidental findings that may come to the attention of the surgical pathologist. In some cases, these may lead to an erroneous diagnosis; in others, they may obscure the diagnosis. The following groups of lesions are discussed: artifacts seen in biopsy and resection material, site-specific changes commonly encountered, incidental findings and structures seen in biopsy and resection material, and age-related changes. A short review of normal histology is included for perspective and a discussion of the abnormal biopsy that appears normal on first review is presented.

Aging↗

Tumor volume: an independent predictor of outcome for laryngeal cancer.

PURPOSE: Other studies have shown a relationship between the volume of laryngeal tumors and local control when treated with radiation therapy. Our purpose was to determine the relationship between tumor volume, clinical staging, and several histologic abnormalities to local control in patients treated with initial surgery. METHOD: Tumor volume was calculated from pretreatment CT scans in 52 patients with squamous cell carcinoma treated surgically. The presence of perineural and lymphatic spread as well as cartilage and vessel invasion were obtained from histology. All cases had at least a 2 year clinical follow-up after initial surgery. Statistical analysis consisted of Mantel-Haneszel chi2-tests and Fisher exact test. RESULTS: Local control rate was 92%. Tumor volume and cartilage invasion were associated with local control (p < 0.05). Local control rate for tumors with volumes of < 16 cm3 was 98% compared with 40% for tumors with volumes of > 16 cm3 (p < 0.05). Evidence of cartilage invasion was associated with increased likelihood of local recurrence. There was no significant association between local control and perineural, vascular, or lymphatic tumor spread. We found a marginally significant association between clinical T-stage and local control (p = 0.05). CONCLUSION: Pretreatment CT volumes are useful in predicting local control in laryngeal carcinoma treated with surgery. Of the histologic features studied, only cartilage invasion was significant in predicting tumor control.

Adult↗

Thymic masses on MR imaging.

MR imaging is an excellent technique for identifying and defining the extent of thymic tumors. T1-weighted spin-echo MR images (e.g., 600/15 [TR/TE]) best demonstrate tumor extent, and T2-weighted images (e.g., 2500/90 [TR/TE]) help differentiate "cystic" from solid thymic masses. Cyst formation and/or hemorrhage appear as areas of high signal intensity (greater than that of fat) on T2-weighted images. Focal areas of low signal intensity (less than that of muscle) correspond pathologically to fibrous capsules and septa, air, or calcification. In this report, the MR appearance of the normal thymus is reviewed briefly, and the gamut of abnormal thymic masses on MR is illustrated.

Adolescent↗

The management of fluid leakage in grossly oedematous legs.

Oedema occurs when there is an abnormal amount of fluid in the interstitial space. Normally about 85 per cent of the fluid that is filtered out of capillaries into this space is reabsorbed; the rest is drained by the lymphatic system (Tortora and Grabowski, 2000). A rise in capillary blood pressure causes increased quantities of fluid to escape from capillaries. As a result, filtration of fluid into the tissues exceeds the reabsorption rate of fluid into the circulation, leading to oedema (Tortora and Grabowski, 2000) (Fig 1).

Bandages↗

[Diarrhea, protein-losing enteropathy and sarcoidosis].

A case of inactive sarcoidosis associated with protein-losing enteropathy, severe hypoalbuminemia and diarrhea is reported. 99mTc-labeled human serum albumin was performed to show protein loss. There was no abnormal upper gastrointestinal mucosa as shown by peroral duodenal and jejunal biopsies. However, a CT scan showed the presence of multiple calcified intra and retroperitoneal lymph nodes, which might explain the presence of lymphatic ectasia and protein-losing enteropathy in this case. A brief literature review of gastrointestinal sarcoidosis is included.

Biopsy↗

[Electron microscopy observation on pre- and post-treatment skin of dorsum pedis of chyluric patient treated with heat-clearing and hemostatic drugs--the lymphangial wall damage being the pathogenesis of chyluria].

Heat-clearing and hemostatic drugs were effective in treating chyluria. In order to explore the curative mechanism, a group of 10 in-patients with chyluria were chosen and treated with heat-clearing and hemostatic drugs for 45 days. Some pre- and post-treatment skin from dorsum pedis of the patients were taken off and observed under electron microscope. It showed that the walls of lymphatic capillaries were seriously damaged before treatment and rehabilitated wall after treatment. The pinocytotic vesicles were packed tightly in the endothelial cells of capillaries before treatment and their number was decreased remarkably after treatment; the mast cells were rare in number and abnormal as spindle shape before treatment and they increased in number and became round or oval in shape, the nucleus became large, the granules they contained became more in number and larger in size after treatment, lymph edema was noticed before treatment and it disappeared after treatment. The result showed that the extensive lesion of lymphangial walls is the pathogenesis of chyluria, while the rehabilitation of them is the curative mechanism. It seems that in the process of rehabilitation the mast cells act took the role of "soldier-engineer".

Adult↗

GLUT1: a newly discovered immunohistochemical marker for juvenile hemangiomas.

Juvenile hemangiomas are common, benign vascular tumors of infancy. These lesions enlarge rapidly through cellular hyperplasia during the first year of life and then involute over several years. Distinctive histopathologic features of hemangiomas diminish during this evolution, and differentiation from vascular malformations becomes increasingly difficult. This distinction has important therapeutic implications, as juvenile hemangiomas differ from malformations in natural history and in potential for recurrence. We report here that high endothelial immunoreactivity for the erythrocyte-type glucose transporter protein GLUT1 is a specific feature of juvenile hemangiomas during all phases of these lesions. In a retrospective study, we found intense endothelial GLUT1 immunoreactivity, involving more than 50% of lesional microvessels, in 97% (139 of 143) of juvenile hemangiomas from patients aged 1 month to 11 years. No endothelial GLUT1 immunoreactivity was found in any of 66 vascular malformations (17 arteriovenous, 33 venous, 11 lymphatic, and 5 port-wine) from patients aged 5 days to 75 years, or in any of 20 pyogenic granulomas or 7 granulation tissue specimens. Abundant Ki-67 positivity in these latter lesions established that GLUT1 expression does not simply reflect mitotically active endothelium. Focal GLUT1 immunoreactivity was found in 3 of 12 angiosarcomas, but not in any of 5 hemangioendotheliomas (epithelioid or infantile kaposiform). These findings establish GLUT1 immunoreactivity as a highly selective and diagnostically useful marker for juvenile hemangiomas. Because high levels of endothelial GLUT1 expression in normal tissue are restricted to microvessels with blood-tissue barrier function, these findings also have implications for the molecular and developmental pathogenic mechanisms of juvenile hemangiomas.

Adolescent↗

[Polyposis of the gastrointestinal tract as a manifestation of diffuse follicular lymphatic hyperplasia].

HISTORY AND ADMISSION FINDINGS: A 21-year-old previously healthy Turkish man who had been living in Germany for 15 years was admitted because of worsening cramp-like abdominal pain with nausea, vomiting and watery diarrhoea. Palpation elicited diffuse muscular guarding over the entire abdomen and a mass of about 8 cm in the right lower abdomen. INVESTIGATIONS: Abnormal laboratory results were erythrocyte sedimentation rate (55 mm), C-reactive protein (6.2 mg/dl), total bilirubin (2.1 mg/dl), creatine kinase (137 U/l) and thymidine kinase (5.5 U/l). There was a slight leucocytosis (13,700/microliter) and mild anaemia (haemoglobin 13.4 g/dl) with a normal differential count. Listeria ivanovii was repeatedly cultured from stool. Ultrasonography and computed tomography of the abdomen demonstrated a 6 cm mass in the right lower abdomen, splenomegaly (15.5 x 5 cm) and several lymphomas, up to 1.8 cm in diameter. Endoscopy revealed dense, in part grass-like, polyps, 3 to 6 mm deep, in the mucosa from the terminal ileum to the rectum, and to a lesser extent also in the duodenum. Histological examination of the polyps demonstrated diffuse follicular hyperplasia without evidence of malignancy. TREATMENT AND COURSE: On antibiotic treatment with ofloxacin (2 x 400 mg intravenously) the symptoms quickly regressed, but the endoscopic findings remained unchanged. CONCLUSION: Diffuse follicular lymphatic hyperplasia manifested itself in this patient as diffuse gastrointestinal polyposis. Listeria ivanovii cannot be ruled out as a causative factor.

Abdominal Pain↗

Minimal immunological changes in structurally malformed rats after prenatal exposure to cyclophosphamide.

In order to compare the sensitivities of morphological and immunological parameters in a teratology study, effects in day 20 rat fetuses were studied after a single exposure to the immunosuppressive cytostatic agent cyclophosphamide (CP) on either day 11 or day 15 of gestation. Teratological methods included evaluation of external and skeletal morphology. Furthermore histology, immunohistochemistry and flow cytometry were performed on fetal thymus, liver and spleen. Immune function was assayed using the Trichinella spiralis infection model. Treatment resulted in dose-dependent gross morphological malformations, and in addition in overt skeletal anomalies such as brachygnathia, wavy ribs, and lordosis. In contrast, the immunological parameters tested revealed only minimal differences between treated and control groups. These results suggest either a remarkable recovery of the immune system after treatment, or a relatively high resistance of the immune system to the present treatment.

Abnormalities, Drug-Induced↗

[Cytogenetic study of acute and chronic leukemias].

From 1983 to 1987 we studied 30 patients with leukemia searching for chromosomal alterations. Cytogenetic studies of bone marrow showed an abnormal karyotype in 19 (67%). Seven out of 14 cases of acute leukemia presented different chromosomic alterations. The Philadelphia chromosome was found in 11 patients with chronic myeloid leukemia and 3 patients with chronic lymphatic leukemia; a Pseudo-Philadelphia chromosome was present in one patient, this being the first such finding in Chile. Thus, the karyotype is a valuable complementary study not only in chronic myeloid leukemia but also in chronic lymphatic leukemia and the acute lymphoblastic and non-lymphoblastic varieties.

Adolescent↗

The nature of mycosis fungoides.

Thirty-four patients with a clinical diagnosis of plaque stage mycosis fungoides, poikiloderma atrophicans vasculare (poikiloderma) and parapsoriasis en plaques (parapsoriasis) were investigated for evidence of extracutaneous disease using a biochemical screen, blood films, bone marrow examination, chest radiograph, abdominal ultrasound, isotope liver scan, liver biopsy and lymphangiography. Skin biopsies were also taken from these patients and, in order to rule out non-specific histological change, from 29 controls with inflammatory skin disease; the slides were examined blind by two independent observers for evidence of mycosis fungoides or poikiloderma and graded accordingly. Both observers were able to differentiate significantly histological grades of mycosis fungoides corresponding to the clinical diagnosis although exact histological grades only corresponded on 35 per cent of slides. Features graded as classical or probable mycosis fungoides were found in between 22 and 67 per cent of patients with parapsoriasis and between 67 and 100 per cent of those with poikiloderma. We found no evidence of extracutaneous disease in any of our patients. Lymphangiograms were abnormal in 30 of 31 examinations but the changes were non-specific and did not correspond to disease type, duration or extent. Four patients had apparently unrelated co-existing disease including chronic lymphatic leukaemia in two, a monoclonal gammopathy and autoimmune haemolytic anaemia. This study shows that in its early stages mycosis fungoides is predominantly if not primarily a cutaneous disease. The findings also suggest that parapsoriasis and poikiloderma are part of the same disorder as mycosis fungoides or evolve into it. Aggressive treatment to prevent progression to mycosis fungoides plaque and tumour stage should therefore be tried. The findings support the idea that mycosis fungoides is a reactive rather than a neoplastic disorder.

Adolescent↗