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Biomedical subjects

J E Fitzpatrick

Publications and source records attributed to J E Fitzpatrick.

At least 37 records · Page 2Linked to original sources

Chronic septic arthritis caused by Borrelia burgdorferi.

Chronic arthritis occurs in 10% of Lyme disease patients. A patient had chronic septic Lyme arthritis of the knee for seven years despite multiple antibiotic trials and multiple arthroscopic and open synovectomies. Spirochetes were documented in synovium and synovial fluid (SF). Polymerase chain reaction (PCR) analysis of the SF was consistent with Borrelia infection. Persistent infection should be excluded with silver stains and cultures in any patient with chronic monoarticular arthritis and a history of Lyme disease.

Adult↗

Calcinosis cutis following electromyographic examination.

We report the first case of calcinosis cutis following electromyographic examination. All previously reported cases of iatrogenic calcinosis cutis occurred following electroencephalographic examination using calcium-containing electrode compounds or following the extravasation of calcium-containing solutions along infiltrated intravenous lines. We also review the literature on calcinosis cutis due to iatrogenic and occupational exposure to calcium salts. The clinical presentation of calcinosis cutis is variable, but the histologic appearance is diagnostic. This entity may be underreported because of its benign, self-limited course and lack of recognition by physicians. Calcinosis cutis should be recognized by family practitioners, dermatologists, and subspecialists who encounter patients who have undergone prolonged monitoring studies that involve the use of electrodes.

Calcinosis↗

Cutaneous macroglobulinosis. A case report with unique ultrastructural findings.

BACKGROUND: Cutaneous macroglobulinosis is a rare cutaneous manifestation of Waldenström's macroglobulinemia. Lesions result from the direct deposition of macroglobulin in the skin and have been called IgM storage papules. A case of cutaneous macroglobulinosis with unique ultrastructural findings was studied. OBSERVATIONS: Cutaneous macroglobulinosis is characterized by multiple flesh-colored papules on extensor skin surfaces. Histologically, there are dermal collections of eosinophilic hyaline material, simulating amyloid. The material is positive on periodic acid-Schiff staining. Amyloid stains are negative or equivocal. Electron microscopy reveals thick, nonbranching, 56-nm-wide, linear material with cross striations at 12-nm intervals. These ultrastructural findings differ from the three previously reported cases. CONCLUSIONS: Cutaneous macroglobulinosis may be a rare presenting sign of Waldenström's macroglobulinemia. Deposits of macroglobulin in the skin result in a histologic picture that greatly resembles amyloid. Histochemical stains, direct immunofluorescence microscopy, and electron microscopy are useful tools that enable accurate diagnosis and help to distinguish cutaneous macroglobulinosis from other deposition disorders.

Humans↗

Microscopic polyarteritis. Report of a case with cutaneous involvement and antimyeloperoxidase antibodies.

BACKGROUND: Microscopic polyarteritis is a systemic small-vessel vasculitis that primarily involves the kidneys but may also affect the skin and other organ systems. This unique vasculitis represents one of the vasculitides with antineutrophil cytoplasmic antibodies, usually of the perinuclear immunostaining pattern (P-ANCA, antimyeloperoxidase antibodies). The objective of this case report is to describe microscopic polyarteritis, the use of antineutrophil cytoplasmic antibodies, and the unique cutaneous histopathologic features of our patient. OBSERVATIONS: We describe a patient with clinical findings consistent with microscopic polyarteritis, the presence of antimyeloperoxidase antibodies, and a specific cutaneous leukocytoclastic vasculitis. This report further characterizes the histopathologic features by demonstrating that the anatomic location of the cutaneous vasculitic lesions is at the level of the dermal arteriolar vessels and postcapillary venules. CONCLUSIONS: Cutaneous arteriolar leukocytoclastic vasculitis may prove to be a histologic hallmark of microscopic polyarteritis when it presents in the skin. However, further case comparisons and histopathologic investigations are needed. The consequences of this systemic vasculitis, if not adequately treated, may be life threatening. Recognition of clinical features, use of antineutrophil cytoplasmic antibodies, and demonstration of a cutaneous arteriolar leukocytoclastic vasculitis may aid in the diagnosis and subsequent treatment of patients followed up by dermatologists for vasculitic ulcers.

Aged↗

Effects of prestorage white cell-reduction of apheresis platelets on platelet glycoprotein Ib and von Willebrand factor.

Twenty plateletpheresis components were harvested from 11 healthy donors and stored in polyolefin bags on a horizontal flatbed agitator at 22 degrees C. After 24 hours, white cells were reduced in one aliquot by centrifugation while the other aliquot was stored unaltered. Samples were obtained aseptically from each of these platelets at intervals for up to 10 days, and measurements were made of platelet glycoprotein Ib (GPIb) by both flow cytometry and polyacrylamide gel electrophoresis, of ristocetin-induced platelet aggregation by impedance aggregometry, and of plasma and platelet von Willebrand factor (vWF) by enzyme-linked immunosorbent assay. Storage of platelets under these conditions was associated with only minor decreases in surface GPIb, intraplatelet vWF, and ristocetin-induced platelet aggregation, and no differences were observed between the white cell-reduced and nonreduced aliquots. No benefit of white cell reduction in such components before prolonged storage is evident in the vWF-platelet interaction.

Blood Component Removal↗

Primary cutaneous adenoid cystic carcinoma treated with Mohs micrographic surgery toluidine blue technique.

A patient with primary cutaneous adenoid cystic carcinoma treated with Mohs surgery is presented. This tumor is characterized clinically by frequent local recurrences and infrequent metastases. Histologically it demonstrates cribiform islands of tumor cells with an abundance of mucin. Because toluidine blue stains this mucin metachromatically, it may be superior to hematoxylin and eosin for identifying the presence of this tumor. We recommend Mohs micrographic surgery with toluidine blue staining technique for the treatment of adenoid cystic carcinoma.

Aged↗

Clinical significance of monoclonal proteins in chronic lymphocytic leukemia.

Serum monoclonal proteins were found in 36 of 111 (32%) chronic lymphocytic leukemia (CLL) patients studied using high-resolution agarose gel electrophoresis combined with immunofixation. In contrast, using immunoelectrophoresis (IEP) and serum protein electrophoresis (SPEP), monoclonal proteins were found in 20 and 4% of CLL patients, respectively. The presence of a monoclonal protein was associated with a median survival of 63 months compared to 103 months for individuals without a monoclonal spike (p < 0.012). This was independent of clinical stage. The data also suggests that the presence of a monoclonal protein may define a group of patients at greater risk for disease progression in the low and intermediate risk groups. (Rai stages, 0, I, II). Further follow-up will be required to determine if this is statistically significant.

Blood Proteins↗

Cutaneous sarcoidal granulomas in a patient with primary biliary cirrhosis.

We report here the case of a fifty-year-old Japanese woman with a history of primary biliary cirrhosis who presented with a sarcoidal granulomatous plaque on the face. Although the occurrence of extra-hepatic granulomas and primary biliary cirrhosis has been documented, this is the first case report of cutaneous sarcoidal granulomas in the absence of demonstrable sarcoidosis occurring in a patient with primary biliary cirrhosis. Because primary biliary cirrhosis and sarcoidosis are systemic granulomatous diseases of unknown cause, the coexistence of findings compatible with both entities has led to speculation that they are related. We provide a brief review of these two diseases to help examine this hypothesis.

Female↗

New histopathologic findings in drug eruptions.

Several new findings associated with hyperpigmentation have been reported in the past decade, the most notable being amiodarone-induced hyperpigmentation, which was shown to demonstrate both a lymphocytic dermatitis as well as yellowish brown granules that can be found within several cell types. The nature of this material has not been elucidated, although the drug or one of its metabolites composes at least a portion of the granules. The nature of the clofazimine-induced hyperpigmentation was shown to be caused by the accumulation of a ceroid lipofuscin within lipid-laden macrophages. Several chemotherapy-induced clinical and histologic changes have been reported in the past decade because of new chemotherapeutic drugs, better recognition of histologic reaction patterns, and the use of higher dosages by oncologists. A unique dermatitis, cutaneous eruption of lymphocyte recovery--although not directly due to use of chemotherapeutic agents--occurs after the return of immunocompetent lymphocytes in the peripheral circulation and skin, producing a maculopapular eruption that demonstrates a nonspecific superficial perivascular dermatitis on biopsy. Another clinically specific reaction, chemotherapy-induced acral erythema, demonstrates a nonspecific histologic pattern characterized by an interface dermatitis. Specific histologic patterns were reported for reactions following use of etoposide--starburst cells--and of busulfan--large atypical keratinocytes. There have been reports of new reactions due to chemotherapeutic agents involving sweat glands: neutrophilic eccrine hidradenitis, characterized by neutrophils and necrosis; and syringosquamous metaplasia, a histologic reaction of the sweat duct characterized by squamous metaplasia. New inflammatory reaction patterns include drug-induced generalized pustular toxic erythema, which histologically shows subcorneal pustules and occasional eosinophils. Cephalosporins were reported to produce a syndrome that clinically and histologically resembles pemphigus. Naproxen is reported to produce a clinical and histologic reaction similar to porphyria cutanea tarda. Quinine and piroxicam both induce a photosensitive dermatitis that histologically shows a nonspecific spongiotic dermatitis. A histologically unique reaction pattern manifesting as a lichenoid giant cell dermatitis may be produced by use of either methyldopa or chlorothiazide. Both phenytoin and carbamazepine produce a dermatitis that histologically imitates mycosis fungoides. Finally, phytonadione injections may produce a clinical and histologic reaction that resembles morphea.

Amiodarone↗

Scar sarcoidosis in pseudofolliculitis barbae.

A man with hilar adenopathy had smooth firm purplish papules (lupus pernio) on his face and pseudofolliculitis barbae (PFB) in his beard area. Biopsies of papules of both the lupus pernio and the PFB revealed non-caseating granulomas consistent with sarcoidosis. This is the first case report of sarcoidosis infiltrating lesions of PFB and the first of lupus pernio co-existing with scar sarcoidosis. In persons with suspected sarcoidosis, enlarged lesions of PFB are sites suitable for confirmatory biopsy. In addition, persons who develop keloid-like papules within their PFB may be providing an early cutaneous clue to undiagnosed systemic sarcoidosis.

Adult↗

Cutaneous angiolipoleiomyoma.

We describe eight cases of cutaneous angiolipoleiomyoma, a rare tumor previously reported only once under the term cutaneous angiomyolipoma. Clinically, the tumors were acquired, solitary, asymptomatic nodules that were always acral in location. Patients' ages ranged from 33 to 77 years (median 52.6 years); the male/female ratio was 7:1. Signs of tuberous sclerosis or renal angiomyolipoma were absent in all cases. Histologically, the tumors were subcutaneous, well circumscribed, and composed of smooth muscle, vascular spaces, connective tissue, and mature fat. In some tumors the fat was the predominant component, and in others smooth muscle predominated. Elastic tissue stains revealed that some blood vessels had developed an elastic lamina whereas other blood vessels lacked it. Additional histologic features occasionally observed included vascular thrombi, glomus bodies, and focal mucin deposition.

Adult↗

Examination of HTLV-I ELISA-positive leukemia/lymphoma patients by western blotting gave mostly negative or indeterminate reaction.

We used enzyme-linked immunosorbant assay (ELISA) and Western blotting, with "purified" human T-cell leukemia virus I (HTLV-I), to test for HTLV-I antibodies in 2583 plasma samples from 1053 leukemia/lymphoma patients treated at Roswell Park Memorial Institute, mostly between 1972 and 1984, and in 110 sera samples from normal healthy persons. The results demonstrate that ELISA and Western blot assay have limitations for HTLV-I antibody detection in an adult T-cell leukemia/lymphoma (ATL) nonendemic population. This conclusion is based on the many false reactives obtained by ELISA, and weak and indeterminate reaction (mostly p19 band) on Western blotting. All moderate to strongly HTLV-I ELISA-positive samples tested were negative for human immunodeficiency virus (HIV) antibodies. Although 6/27 mycosis fungoides (MF) patients tested gave mostly a weak reaction on HTLV-I ELISA, 3/6 MF patients gave multiple bands (p19, p31, p36, gp46) on Western blotting and three samples from one patient gave the same p31, p36, and gp46 bands. This may suggest involvement of some HTLV-I-related virus in MF. These results also indicate that prevalence of HTLV-I infection in leukemia/lymphoma patients was rare, if it exists at all, since, despite the reactivity of some sera with HTLV-I-suspected antigens, none of the samples satisfy the USPHS criteria for positivity which is based on the detection of antibodies to gag protein p24 and to an env gene product gp46 or gp61/68.

Adolescent↗

Ristocetin-induced platelet aggregate formation and adherence to the probe of an impedance aggregometer.

The examination of the interaction between ristocetin and platelets generally is performed in an optical aggregometer where aggregate formation is detected by a change in turbidity. Ristocetin also will induce conductivity changes in an impedance aggregometer with either fresh whole blood or fresh platelet-rich plasma due to aggregate attachment to the electrical probe. Formaldehyde-fixed platelets, however, do not attach, and although fresh platelets agglutinate at extremes of pH, probe adhesion does not occur unless the pH is within a certain range. Incubation of platelet preparations with neuraminidase, colchicine, or cytochalasin-b affects neither aggregate formation nor probe adherence. Incubation of platelet preparations with a fibronectin tetrapeptide inhibitor eliminates aggregate formation in response to collagen but is without effect on ristocetin-induced attachment of aggregates to the impedance probe. It is suggested that the ability of platelet aggregates to attach to an impedance probe in response to ristocetin is dependent on additional aspects of the functional integrity of platelet preparations and that these changes required for probe adherence may have in vivo significance.

Blood Platelets↗

Diisopropylfluorophosphate binding proteins (serine hydrolases) from normal and leukemic hematopoietic cells.

Normal and leukemic hematopoietic cell lysates were labeled with [3H]-diisopropylfluorosorophosphate ([3H]-DFP), an active site inhibitor of serine hydrolases. The labeled proteins in the lysates were examined by sodium dodecyl sulfate-polyacrylamide gel electrophoresis followed by counting of gel segments for radioactivity. The results indicate the presence of distinct [3H]-DFP binding patterns for different normal and leukemic hematopoietic cells; significantly lower labeling in normal or leukemic lymphoid cells compared to myeloid or monocytoid cells; lower labeling in acute myeloblastic leukemia (FAB-M1) as compared to acute myelomonocytic leukemia (FAB-M4), chronic myelomonocytic leukemia or monocytes and an increase in [3H]-DFP binding with cell maturation along granulocytic series. Thus, these patterns could be useful in discriminating acute lymphoblastic leukemia from myeloid/monocytoid types of leukemia and for following maturation of myeloid cells, and perhaps for studying functional or maturation defects in hematopoietic cells in other pathological conditions.

Blood Donors↗

Whole blood aggregation in von Willebrand disease.

Platelet function testing in von Willebrand disease is generally performed in platelet rich plasma using an optical system. The characteristic abnormality is an abnormal response to the agglutinating agent, ristocetin. Platelet aggregation can be also studied in whole blood using either an impedance aggregometer or a particle counter. Fifteen patients with von Willebrand disease and fifteen normal subjects were studied using both whole blood methods. In the impedance system, normal subjects responded to ristocetin (1 mg/ml) with short lag phases (less than 70 sec) and normal maximum aggregation greater than 5 omega). Only three patients with von Willebrand disease responded with normal maximal aggregation but each of these had a prolonged lag phase, and this may be a useful diagnostic parameter. In the particle counter, discrimination between normals and some von Willebrand disease patients was possible but an overlap between normals and von Willebrand patients is evident. It is concluded that the platelets in patients with von Willebrand disease exhibit the same abnormalities in whole blood as in platelet rich plasma and that the combination of impedance aggregometry and a factor VIII procoagulant assay is a time-efficient and sensitive method to screen for von Willebrand disease.

Humans↗

Serum levels of cancer-associated antigen CA-195 in gastrointestinal cancers and its comparison with CA19-9.

The antibody against CA-195 binds to an epitope that consists of both Lewis A and sialylated Lewis A blood group antigen, whereas CA19-9 has shown specificity for sialylated Lewis A blood group antigen. CA19-9 and CA-195 levels were measured in the sera of 52 normal subjects; 65 benign disease patients; and 74 non-gastrointestinal, 149 colorectal, and 119 upper gastrointestinal cancer patients to correlate their levels with disease status of the patients. Low incidence and levels were found among normal subjects for both markers; however, in the benign disease group a slightly higher incidence of elevation was seen for CA19-9. Among colorectal cancer patients CA-195 appeared to show higher sensitivity for primary as well as advanced disease. Levels of both markers showed similar incidences of elevation among upper gastrointestinal cancer patients. Based on these results the contribution of Lea specificity of CA-195 cannot be ruled out, and it may be used alone or in combination with other markers for monitoring of patients with colorectal, pancreatic, gastric, gall bladder, bile duct, and liver cancers.

Antigens, Tumor-Associated, Carbohydrate↗