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W D Travis

Publications and source records attributed to W D Travis.

At least 181 records · Page 10Linked to original sources

Systemic mast cell disease. Analysis of 58 cases and literature review.

Based on study of 58 histologically proved cases of SMCD, we believe that the prognosis of most SMCD patients can be anticipated at the time of initial diagnosis by using 5 independent significant predictors developed in a multivariate model. Our study confirms the significance of several previously reported poor prognostic factors: absence of skin involvement and the presence of hepatosplenomegaly, cytologic atypia, and a hypercellular bone marrow. However, in contrast to previous reports we did not find a uniform correlation between the presence or absence of skin involvement and prognosis. The observation that anemia was strongly related to so many prognostic variables may be due to the number of patients in our study with associated hematologic disorders. Alternatively, this evidence of ineffective erythropoiesis may support the concept that SMCD is a myeloid stem cell disorder and frequently affects other hematopoietic cell lines. The observation that death occurs within the first 3 years in most fatal cases of SMCD suggests that these patients should be followed carefully for this interval after initial diagnosis, especially if poor prognostic features are present. Currently there is no curative therapy for SMCD.

Female↗

Isolation and sequence analysis of amyloid protein AA from a patient with cystic fibrosis.

This study represents the first sequence analysis of an amyloid fibril protein from a patient with cystic fibrosis. Although chronic infections are a hallmark of cystic fibrosis, secondary amyloidosis is a rare complication, and during the past 20 years, only 16 cases of amyloidosis in patients with cystic fibrosis have been reported. We examined amyloid fibrils isolated from the spleen of a 25-year-old man who had a history of a chronic cough since infancy and a diagnosis of cystic fibrosis at age 6 years. After solubilization in 6 mol/L guanidine and purification by gel filtration, the major component of the amyloid fibrils was a homogeneous 8000 dalton protein that reacted positively with antiserum to human amyloid A (AA) protein. Complete protein sequence analysis was carried out by using the whole protein and fragments obtained by treatment of the protein with cyanogen bromide, lysyl endopeptidase, and carboxypeptidase. The protein contained 76 residues and showed minor heterogeneity when compared with other AA protein sequences. The cystic fibrosis AA protein represents a product of the SAA-specific cDNA clone now known to be the alpha-allelic form of SAA1 in which valine is present at position 52 and alanine is at position 57.

Adult↗

Pathology of the lymph node and spleen in systemic mast cell disease.

The spleen and lymph node are two of the most common organs involved in systemic mast cell disease (SMCD). However, SMCD infiltrates in the spleen and lymph node have a broad spectrum of morphological patterns which can make it difficult to recognize the diagnosis, especially when specimens are examined from patients in whom SMCD is not suspected. We reviewed the pathological features of 16 spleen and 23 lymph node specimens from 19 patients which represented all available material from a series of 58 Mayo Clinic patients with SMCD. The purpose of this study was to investigate the pathological manifestations of SMCD involvement in the spleen and lymph node and to address difficulties in differential diagnosis. All compartments of the spleen and lymph node were found to be affected by SMCD. SMCD lesions in the spleen were found in a paratrabecular (92%), parafollicular (69%), follicular (15%), and a diffuse red pulp (8%) distribution. In the lymph node, mast cell infiltrates affected the paracortex (88%), the parafollicular region (50%), the follicles (25%), the medullary cords (13%), and the sinuses (6%). Mast cells were frequently found in a perivascular location, and associated eosinophilia was common. Because of the broad spectrum of histological manifestations of SMCD in the spleen and lymph node, a wide range of differential diagnoses is discussed including follicular lymphoma, T-cell lymphoma, monocytoid B-cell hyperplasia and lymphoma, Kaposi's sarcoma, and Langerhans' cell granulomatosis.

Diagnosis, Differential↗

Megakaryoblastic transformation of chronic granulocytic leukemia.

Three cases of megakaryoblastic transformation of chronic granulocytic leukemia (CGL) are reported. In each case, the leukemic transformation had morphologic features suggesting megakaryocytic differentiation. This was confirmed by positive immunostaining with a monoclonal antibody (HP1-1D) specific for platelet and megakaryocyte glycoprotein IIb/IIIa antigen, which was expressed by the majority of the leukemic blasts in all three cases. Cases with evidence of multilineage differentiation of the leukemic transformation were excluded. A striking feature in two patients was the manifestation of lytic bone lesions and soft tissue masses at presentation. A biopsy of a lytic bone lesion and soft tissue mass in one patient revealed a megakaryoblastic leukemic infiltrate, which by immunocytochemical staining was positive for the megakaryocytic markers, glycoprotein IIb/IIIa antigen, and Factor VIII (von Willebrand factor) antigen. In contrast to granulocytic sarcomas, the megakaryoblastic sarcoma did not stain cytochemically for chloroacetate esterase. The mean survival after acute transformation was 5.3 months. The three cases of megakaryoblastic transformation represented a significant proportion of all CGL blastic transformation cases (ten cases) evaluated by bone marrow examination in our institution during a 13-month period. Megakaryoblastic transformation of CGL may occur more frequently than has been appreciated, and can present as lytic bone lesions or as soft tissue megakaryoblastic sarcomas.

Adult↗

Primary extranodal soft tissue lymphoma of the extremities.

Since true primary lymphomas of soft tissues are extremely rare and there are few well-documented cases in the literature, we report eight cases to help define the clinical and pathologic features of this type of extranodal lymphoma. Among 7,000 malignant lymphomas seen at Mayo Clinic over a 10-year period, we found eight cases of Stage IAE extranodal malignant lymphoma that presented as a soft tissue mass in the upper or lower extremities. The tumors occurred in the following sites: thigh (n = 4), upper arm (n = 2), ankle (n = 1), and calf (n = 1). Two cases had low-grade histology: small lymphocytic lymphoma and follicular mixed small-cleaved and large-cell lymphoma. An intermediate-grade histology was present in two cases: diffuse mixed small- and large-cell lymphoma and diffuse large-cell (cleaved) lymphoma. Half of the patients had a high-grade lymphoma: diffuse large-cell (n = 3) and large-cell, immunoblastic (n = 1). The results of immunohistochemistry and electron microscopy in selected cases are discussed in the context of the differential diagnosis with other small, round-cell cancers. Four patients died of recurrent lymphoma, one at 7 months, two at 15 months, and one at 4 years. Three patients are alive without evidence of disease at 3, 8, and 13 years. One patient is alive and is receiving chemotherapy for locally recurrent lymphoma 6 months after diagnosis.

Adult↗

Diffuse pulmonary hemorrhage. An uncommon manifestation of Wegener's granulomatosis.

We report two cases of Wegener's granulomatosis with the unusual manifestation of diffuse alveolar hemorrahge. One patient with well-documented Wegener's granulomatosis developed alveolar hemorrhage 4 weeks after leukopenia necessitated the discontinuation of cyclophosphamide. The second patient presented with pulmonary hemorrhage and died 10 days after an open-lung biopsy in which histologic features of Wegener's granulomatosis were overshadowed by alveolar hemorrhage. Lung biopsies in both cases showed marked alveolar hemorrhage and pulmonary capillaritis. The importance of recognizing capillaritis and other subtle histologic features of Wegener's granulomatosis are emphasized.

Aged↗

Mast cell leukemia: report of a case and review of the literature.

We report the clinical and pathologic findings in one case of mast cell leukemia observed in a series of 60 patients with systemic mast cell disease. The leukemic variant of systemic mast cell disease is rapidly fatal (mean duration of survival, less than 6 months) in contrast to most nonleukemic cases, which follow an indolent clinical course. On the basis of our case and eight previously reported cases, mast cell leukemia is characterized by a substantial increase in atypical mast cells in the peripheral blood, diffuse infiltration with atypical mast cells in the bone marrow, a strong association with peptic ulcer disease, prominent constitutional symptoms, and hepatosplenomegaly. These cases should be distinguished from malignant mastocytosis without a substantial number of circulating atypical mast cells and also cases of acute nonlymphocytic leukemia that arise in the background of systemic mast cell disease.

Biopsy↗

Secondary (AA) amyloidosis in cystic fibrosis. A report of three cases.

The authors report the pathologic features of three cases of amyloidosis associated with cystic fibrosis. Renal biopsy led to the diagnosis (case 1) or suspicion (case 2) of amyloidosis in patients who were 23 and 21 years old, respectively. The third patient died at age 22 years, and amyloidosis was not discovered until autopsy. Immunohistochemical staining and potassium-permanganate pretreatment of histologic sections in all three cases provided evidence that the amyloid seen in these patients is of the secondary (AA) type. Congo red staining in each case and electron microscopy in case 1 confirmed the initial diagnosis of amyloidosis. A markedly elevated serum amyloid A protein (160 micrograms/mL; normal less than 1 microgram/mL) in case 1 indicated the presence of large quantities of the precursor protein from which the AA fibrils of secondary amyloid are derived. The kidneys, spleen, and liver contained amyloid deposits in autopsy material from all three cases. Involvement of other organs by amyloid was variable. Review of autopsy material in Boston from 23 additional cystic fibrosis patients with long-term survival did not reveal any evidence of amyloidosis. It appears that secondary amyloidosis is emerging as a significant, although rare, complication of cystic fibrosis as greater numbers of these patients survive into adulthood.

Adult↗

Saddle embolism of the aorta. A complication of streptokinase therapy.

Streptokinase was given to a patient with an unsuspected left ventricular aneurysm as treatment for acute pulmonary embolism. After 2 days of therapy, a large thrombus dislodged from the left ventricular aneurysm and produced an acute saddle embolic occlusion of the abdominal aorta. Detection of mural thrombi by two-dimensional echocardiography in patients with evidence of previous myocardial infarction might prove helpful in identifying those at risk for this complication of streptokinase therapy.

Embolism↗

Immunostaining for hemoglobin in two cases of myospherulosis.

To investigate the previously reported origin of myospherulosis from altered erythrocytes, we used an antibody to hemoglobin. Positive staining further supports the origin of myospherulosis from red blood cells. Immunostaining for hemoglobin provides a practical method for distinguishing myospherulosis from other spherulelike structures.

Adult↗

Silicone-induced endocarditis. A complication of transvenous cardiac pacing catheterization.

We report a case of silicone-induced endocarditis as a newly recognized complication of transvenous cardiac pacing catheterization. Silicone was found embedded in thrombotic vegetations on the tricuspid valve of a patient who had a long-standing pacing catheter in the right side of his heart. Scanning electron microscopy and energy-dispersive x-ray analysis confirmed the presence of silicone in the vegetations and in the catheter tip.

Aged↗

Q fever. A clinicopathologic study of five cases.

Bone marrow and liver biopsy specimens from five patients with documented Q fever were reviewed. Eight bone marrow and two liver specimens had been obtained from eight days to two months after the onset of symptoms in the five patients. Three had Q-fever hepatitis; one had Q-fever endocarditis. The classic "doughnut" granulomas of Q fever were present in either the liver or initial bone marrow biopsy in all four of the untreated patients. It was recognition of the classic granulomas that prompted serologic studies for Q fever in three of the four patients.

Adult↗

Retroperitoneal germ cell tumors in childhood. A clinical and pathologic study of 11 cases.

During the last 54 years at the Children's Hospital 11 children (10 female, 1 male) had been treated for an extragonadal germ cell tumor arising in the retroperitoneum. There were eight teratomas (five mature, three immature), two endodermal sinus tumors and one tumor with a mixture of both components. Abdominal pain or discomfort was the usual presenting complaint, with the average age at diagnosis being 18 months. On physical examination, each child had a palpable abdominal mass usually localized in the upper quadrants. The finding of calcification, bone or teeth, on radiologic study was most helpful in establishing a preoperative diagnosis of teratoma. The preferred treatment for children with mature and immature teratomas is complete surgical resection; decisions regarding adjuvant therapy for patients with immature tumors must be evaluated on an individual basis. The prognosis remains guarded for children with unresectable primaries or those with endodermal sinus tumor. Of three tumor-related deaths, two were due to metastatic endodermal sinus tumor and one resulted from extensive local growth by immature teratoma. Successful management of children with endodermal sinus tumor requires surgery aimed at debulking or complete resection in combination with aggressive adjuvant chemotherapy.

Child, Preschool↗

Silicone granulomas: report of three cases and review of the literature.

Since silicone is rapidly becoming one of the most commonly used biomaterials in modern medicine, pathologists will be observing increasing numbers of cases of silicone-related disease. Although numerous case reports have established that silicone elicits a characteristic response in tissues, the varying tissue reactions to silicone gels, liquids, and elastomers (rubber) have not been emphasized. Three cases are reported, and the literature is reviewed to illustrate the varying features of tissue reaction to silicone in its different forms. The first case is an example of silicone lymphadenopathy in an inguinal lymph node. This case demonstrates exuberant foreign body granuloma formation in response to particles of silicone elastomer. The second case involves a patient who had facial subcutaneous liquid silicone injections, and the third case is that of a woman in whom breast carcinoma developed 13 years after mammary augmentation with liquid silicone injections. These two cases illustrate the characteristic reaction to silicone liquid, with numerous cystic spaces and vacuoles in the soft tissues but minimal or no foreign body giant cell reaction. Scanning electron microscopy and energy dispersive x-ray analysis were performed in the first two cases, confirming the presence of silicon. Silicone migration and the clinical significance of various silicone-induced lesions are discussed.

Adult↗

Benign vascular invasion in vasitis nodosa.

Vasovasostomy specimens were examined for vascular changes in 50 cases of vasitis nodosa. In five, benign gland- or ductule-like structures were seen invading small veins and, in one, also the small arteries. The intravascular ductules appeared benign, and there was no clinical evidence of malignancy. The presence of ductules in the blood vessels always was accompanied by marked elastosis. In 12 additional cases, elastosis of arteries and veins was seen without intravascular ductules. These changes suggest that proliferating ductules in vasitis nodosa invade the blood vessels after they have become obliterated by regressive and reparative processes.

Adult↗

Adult-onset urticaria pigmentosa and systemic mast cell disease.

The records of 32 patients with adult-onset urticaria pigmentosa were analyzed to determine if any clinical or pathologic findings could distinguish urticaria pigmentosa associated with systemic mast cell disease from urticaria pigmentosa with no clinical evidence of systemic mast cell disease. Thirteen patients had biopsy-proven systemic mast cell disease, and 19 had no documentation of systemic mast cell disease after at least 20 years of follow-up. Generally, urticaria pigmentosa with systemic mast cell disease could not be differentiated from urticaria pigmentosa with skin involvement only. Although most patients in both groups had mast cells in a perivascular location, four patients had dense infiltrates filling the papillary dermis. Two of these patients had biopsy-proven systemic mast cell disease, and two had clinical symptoms that could have been produced by systemic mast cell disease, although the diagnosis was not proved by biopsy. The presence of dense infiltration by mast cells in urticaria pigmentosa with cytologic atypia may correlate with the presence of systemic mast cell disease.

Adult↗

The histopathologic spectrum in Mycobacterium marinum infection.

Review of nine culture-positive cases of Mycobacterium marinum infection revealed a broad range in the histopathologic features of lesions produced by this organism. Four synovial lesions and five cutaneous infections were observed. A range of inflammatory changes were seen in both synovial and skin lesions, varying from mostly acute inflammation with suppuration to a more chronic process with numerous, well-formed granulomas. Organisms were observed in the biopsy sections of only one of the nine cases. Therefore, culture of the biopsy tissue at 30 degrees C is crucial in establishing the diagnosis. These cases emphasize the importance of considering mycobacterial infection and performing cultures even when granulomatous changes in the synovium or skin are subtle.

Adult↗

The frequency of perineurial ductules in vasitis nodosa.

In a retrospective histologic study, 154 vasovasostomy specimens (77 patients) yielded 50 examples of vasitis nodosa. These cases were examined for nerve invasion by proliferating ductules; benign neural invasion was present in four instances, and four additional cases were found on step sectioning the vasovasostomy specimens, totaling eight cases (16%) of the 50 studied. Usually only one or two nerves were invaded. The number of glands within individual nerves varied from one to eight. The findings are discussed in the context of benign glandular invasion of nerves in other locations.

Adult↗