Thrombocytopenia caused by splenic hamartoma: resolution after splenectomy.

We report a rare case of isolated thrombocytopenia due to a large hamartoma of the pulposal type, which resolved with splenectomy in a 46-year-old man. Although hamartomas are usually found incidentally, they may cause hematologic disturbances such as thrombocytopenia, anemia, or splenic rupture. The diagnosis of splenic hamartoma is difficult to make preoperatively. Splenectomy is important in excluding malignant tumors from the diagnosis when the etiology of a splenic mass is unclear and ameliorating hematologic symptoms related to hamartomas.

Perception of the nurse practitioner's role and job satisfaction in the United States Navy.

A few studies have specifically examined the role perception and job satisfaction of civilian nurse practitioners (NPs), but no data exist for U.S. Navy NPs. The purpose of this study was to describe the perceptions of the Navy NP's roles by commanding officers, directors of nursing services, physicians, and NPs, and the level of job satisfaction among Navy NPs. A total of 907 questionnaires investigating NP role and job satisfaction were mailed to 29 Naval hospitals and clinics in the United States and overseas. The sample size totaled 450 (50% response rate). Of the 450 respondents, 45 NPs also filled out the Measure of Job Satisfaction survey. There were measurable differences in NP role perception by the sample groups. Role conflict and ambiguity were shown. Autonomy, sense of accomplishment, and time spent in patient care were major satisfiers, whereas pay, overall staffing levels, time available to get through work, and the amount of time spent on administrative work were the major dissatisfiers.

Cardiopulmonary pathology in patients with sleep apnea/obesity hypoventilation syndrome.

We reviewed clinical data, autopsy reports, and microscopic slides on 10 patients with sleep apnea/obesity hypoventilation syndrome (SA/OHS) to define the cardiopulmonary pathological features and establish clinicopathologic correlations. Ten obese (>136 kg) patients without SA/OHS were studied as controls. Patients with SA/OHS exhibited biventricular cardiac failure and pulmonary hypertension with a higher prevalence of moderate/severe pulmonary hemosiderosis (8 v 0 patients), alveolar hemorrhage (7 v 4 patients), capillary proliferation (4 v 0 patients), iron encrustation of elastica (1 v 0 patients) and medial hypertrophy of muscular pulmonary arteries (11.9 +/- 2.4 v 9.7 +/- 1.6%) (P < .05). In two patients capillary proliferation resembled capillary hemangiomatosis. Mean right ventricular thickness was higher in the SA/OHS group (0.71 +/- 0.17 v 0.42 +/- 0.1 cm) (P < .01). Four patients with SA/OHS and three controls had moderate/severe myocardial fibrosis. Biventricular cardiac failure caused death in seven patients with SA/OHS. Hypoxia is probably the most important cause of pulmonary hypertension, arterial muscularization, and right ventricular hypertrophy in SA/ OHS. Left ventricular failure in some SA/OHS patients may be the result of hypertensive cardiac disease. In others, the etiology of left ventricular failure was not determined morphologically, suggesting functional abnormalities related to obesity and/or apneic episodes.

Recurrent acute scleroderma renal crisis complicated by thrombotic thrombocytopenic purpura.

Acute renal crisis as an early manifestation of scleroderma is underemphasized, and its recurrence after initial successful therapy is rare. We describe a 32-year-old woman who presented with scleroderma renal crisis. A second episode of apparent renal crisis, however, was complicated by thrombotic thrombocytopenic purpura, which led to pancreatitis, a large cerebral infarction, and fatal outcome despite intensive therapy. This case illustrates the complexity and severity of diffuse systemic sclerosis presenting with multiple, major organ complications.

Psoriatic spondyloarthropathy with secondary amyloidosis.

We report an HLA-B27 positive patient with severe psoriatic spondyloarthropathy who developed secondary reactive amyloidosis. The clinical course was characterized by progressive renal failure with nephrotic syndrome and cardiac dysfunction. He died suddenly and autopsy revealed diffuse amyloid deposits in the heart, kidneys, and many other organs. Amyloid deposits were present in the cardiac conduction system including the sinoatrial and atrioventricular nodes, myocardial interstitium, and intramyocardial small blood vessels. The severe AA amyloid deposit in the heart with involvement of conduction system is unusual, and has not been described in patients with psoriasis or psoriatic arthritis.

Inflammatory pseudotumor of the spleen: a rare entity that may mimic hematopoietic malignancy.

A case of benign inflammatory pseudotumor of the spleen is described in a 71-year-old man who presented with left upper quadrant pain and anemia. Definitive diagnosis was made by a combination of microscopic examination with immunohistochemical stains and flow cytometry studies. To our knowledge this is only the 24th reported case of inflammatory pseudotumor involving the spleen. Recognition of this rare entity is important because the clinical manifestations and radiographic features may be indistinguishable from a malignant lymphoproliferative disorder. The clinical presentation, radiographic findings, gross and microscopic features, results of surgery, and long-term follow up are reviewed for our case and the previously reported cases in the literature.

The Kleihauer-Betke test. Clinical utility, indication, and correlation in patients with placental abruption and cocaine use.

This study examines the indications for performing the Kleihauer-Betke (KB) test and makes recommendations for its use. Results of 523 KB tests performed during 1993 at our hospital (Cleveland, Ohio) are reviewed in conjunction with surgical pathology reports of placental findings, obstetric records, and toxicology results. We conclude that the KB test should be performed following a positive screening test on all Rh negative mothers of Rh positive infants. Additional indications include cases of maternal trauma, unexplained increased maternal alpha-fetoprotein levels, fetal distress with abnormal heart tracings, intrauterine fetal death, and cases of unexplained neonatal anemia. We note that the KB test should not be performed to detect suspected placental abruption.

Rounded atelectasis and fibrotic pleural disease: the pathologic continuum.

Although rounded atelectasis was recognized in 1928, its relationship to fibrotic pleural disease almost has gone unnoticed. We present four cases of lobar rounded atelectasis that help to clarify this relationship. Rounded atelectasis is part of the spectrum of fibrous pleural disease. Any portion of lung may become entrapped by the pleural process. When focal areas are involved rounded atelectasis results. When a larger portion of the thorax is involved with this process, lobar rounded atelectasis may occur. Very extensive disease is recognized as fibrothorax.

Immunofluorescence studies of lung tissue in cystic fibrosis.

Previous studies have suggested that immune mechanisms contribute to lung injury in cystic fibrosis (CF); however, there have been no comprehensive studies of immunofluorescent staining patterns in CF lung tissue. We performed immunofluorescence (IF) studies for immunoglobulins, C3, and fibrinogen on autopsy frozen lung tissue from 21 CF patients. Results were compared with lung findings in patients without CF. In CF-derived lung tissue fibrinogen was ubiquitous along the alveolar wall, alveolar space, and interstitium. Free immunoglobulin G (IgG) and IgA coated the alveolar surface segmentally in 14 and 6 cases, respectively. Unequivocal interstitial deposits were infrequent and IgM was present in blood vessels in one patient only. Intra-alveolar and interstitial inflammatory cells demonstrated cytoplasmic IgG, IgA, and IgM, respectively, in 18, 14, and 6 patients. C3 was seen only segmentally along the alveolar wall in two patients and in blood vessels in one. Antinuclear antibody (ANA) staining of interstitial cells for C3 and immunoglobulins was seen in five patients, four of whom had interstitial pneumonitis. Insignificant amounts of alveolar or interstitial fibrinogen and immunoglobulins in inflammatory cells were seen in controls in the absence of lung inflammation. The IF patterns were similar in the inflammatory lesions of CF and control specimens. The IF patterns observed in CF lung tissue are consistent with nonspecific vascular leakage and chronic inflammation with little evidence of immune complex deposition in the interstitium or blood vessels. This study confirms previous reports of ANA activity in CF patients, although the significance of this finding is unknown.

Recurrent pericarditis and cardiac tamponade in a patient with hypocomplementemic urticarial vasculitis syndrome.

We describe a patient with hypocomplementemic urticarial vasculitis syndrome complicated by recurrent pericarditis and cardiac tamponade. The episodes of pericarditis were associated with urticarial vasculitis, hypocomplementemia, and circulating IgG antibodies to the collagen-like region of Clq. Histopathologic examination of the skin and pericardium demonstrated vasculitis associated with immunoglobulin and complement deposition suggesting an immune complex mediated etiology. Recurrent pericarditis should be included in the clinical spectrum of hypocomplementemic urticarial vasculitis syndrome.

Intravascular papillary endothelial hyperplasia of superior vena cava: a rare cause of the superior vena cava syndrome.

The superior vena cava syndrome associated with an intraluminal vascular proliferative lesion is extremely rare. A case of intravascular papillary endothelial hyperplasia of the superior vena cava causing obstructive symptoms is reported that was successfully managed by surgical excision.

Bilateral intrapulmonary hematomas.

A 67-yr-old man, known to have chronic obstructive lung disease, developed bilateral localized pulmonary densities on chest radiographs after cardiopulmonary resuscitation. An autopsy disclosed bilateral intrapulmonary hematomas without communication with bronchi, pulmonary arteries, or pleural cavities. We suggest blunt pulmonary injury is the most probable cause of the hematomas and discuss its pathogenic mechanism. Intrapulmonary hematomas should be considered in the differential diagnosis of pulmonary densities developing after a vigorous resuscitation.

Acalculus lymphoeosinophilic cholecystitis associated with interleukin-2 and lymphokine-activated killer cell therapy.

A case of unusual cholecystitis that developed on completion of interleukin-2 and lymphokine-activated killer cell therapy is described. A 62-year-old man was treated with interleukin-2 and lymphokine-activated killer cells for disseminated renal cell carcinoma. During the course of the immunotherapy, his serum alkaline phosphatase level increased, as did the peripheral eosinophil count (0.31). Subsequently, clinical and radiologic evidence of acute cholecystitis was noted. The removed gallbladder showed acalculus cholecystitis with extensive diffuse infiltrates of numerous eosinophils and T lymphocytes, but sparse polymorphonuclear leukocytes. The authors name this unusual cholecystitis acalculus lymphoeosinophilic cholecystitis and believe it to be associated with interleukin-2 and lymphokine-activated killer cell therapy. The pathogenic relationship is discussed.

Shrinking pleuritis with lobar atelectasis, a morphologic variant of "round atelectasis".

Round atelectasis (shrinking pleuritis) is typically a localized process characterized by focal pleural scarring and subjacent peripheral atelectasis. We report three patients, studied at autopsy, with an unusual variant of round atelectasis, termed shrinking pleuritis with lobar atelectasis, which is characterized by lobar atelectasis, visceral pleural fibrosis involving multiple lobes, interlobar fibrous cords, pleural effusion, and nonspecific, persistent infiltrates on chest radiogram. The possible causes of shrinking pleuritis with lobar atelectasis in our patients were multiple and included environmental dust exposure, infection, uremia, and recurrent pleural effusions. Our findings support both the folding (pleural effusion) and fibrosing (pleural injury) theories of pathogenesis of round atelectasis and emphasize the spectrum of morphologic variability in this condition.

Acquired cystic disease of the kidneys and renal cell carcinoma in chronic renal insufficiency without dialysis treatment.

We report 3 cases of acquired cystic disease of the kidneys with associated renal carcinoma in 2 of the cases. In all 3 cases, the patients had chronic renal insufficiency due to hypertension but had never required dialysis. Review of 176 reported cases of acquired cystic disease of the kidneys and renal tumors disclosed that 18 patients (including 1 previously reported by us) had never received dialysis treatment. These cases support the hypothesis that acquired cystic disease of the kidney is not restricted to patients treated with maintenance dialysis. Among the 18 patients, hypertension was the most common underlying cause of renal failure. Patients with chronic renal failure due to or associated with severe hypertension should be monitored carefully for the development of both renal cysts and tumors even though they have not started on chronic dialysis.