Charles M. Lombard

A clinicopathologic study of 34 cases of diffuse pulmonary hemorrhage with lung biopsy confirmation

Based on a clinicopathologic study of 34 patients with biopsy-confirmed diffuse pulmonary hemorrhage (DPH). we present an approach to the differential diagnosis of DPH with attention to histologic features such as capillaritis and the importance of laboratory tests such as anticytoplasmic autoantibodies (ACPA). The following DPH syndromes were encountered: antibasement membrane antibody (ABMA) disease (four cases); idiopathic pulmonary hemorrhage (four cases); Wegeners granulomatosis (WG) (five cases); probable WG (six cases); systemic necrotizing vasculitis otherwise unclassified (three cases); systemic lupus erythematosus (two cases); rheumatoid arthritis (one case); seronegative juvenile rheumatoid arhritis (one case); IgA nephropathy (one case); idiopathic glomerulonephritis (two cases—one with and one without immune complexes); and unclassified pulmonary-renal syndromes (five cases). Capillaritis was found in lung biopsy samples from 30 of the 34 patients (88%) and included patients with every type of DPH syndrome. Serologic testing for ACPA was useful in the diagnosis of WG. Identification of ABMA in the serum, kidney, or lung was the defining feature for the diagnosis of ABMA-mediated disease. Subclassification of the cases could not be done solely on histologic grounds except for cases of WG that showed granulomatous inflammation, foci of necrosis, or vasculitis. Classification of the remaining cases required correlation with (a) clinical and laboratory data; (b) biopsy samples from other sites such as the kidney, nasal sinuses, or skin; and (c) results of immunofluorescence or electron microscopy of kidney or lung biopsy samples.

Histiocytosis X in the lung

The histologic and clinical features of pulmonary histiocytosis X are discussed. The majority of patients are in the third and fourth decades of life. They present with nonspecific respiratory complaints and bilateral reticulonodular infiltrates that can be seen on chest radiographs. The histologic lesions of pulmonary histiocytosis X are scattered, discrete nodules that frequently center on small airways. The initially cellular nodules are replaced progressively by fibrous tissue. The cell infiltrates in active lesions contain variable numbers of eosinophils and histiocytes resembling Langerhans cells of the epidermis (Hx cells). In most patients the disease resolves or stabilizes, leaving few, if any, significant residues. A minority of patients develop progressive pulmonary disease that is ultimately fatal. The cause of pulmonary histiocytosis X is unknown, but two recent series suggest a relationship to cigarette smoking.

Visceral bacillary epithelioid angiomatosis: Possible manifestations of disseminated cat scratch disease in the immunocompromised host: A report of two cases

Opportunistic infection with the causative agent of cat scratch disease may be responsible for an unusual vascular proliferative lesion, referred to as bacillary epithelioid angiomatosis, previously described only in human immunodeficiency virus (HIV)-infected patients. We present a case of an HIV-infected patient with bacillary epithelioid angiomatosis involving the liver and bone marrow causing progressive hepatic failure. We also report a case of a cardiac transplant recipient with hepatic and splenic bacillary epithelioid angiomatosis manifesting as a fever of unknown origin, a previously unreported event in a non-HIV-infected patient. These cases represent the first documentation of bacillary epithelioid angiomatosis with visualization of cat scratch-like organisms involving internal organs.

Immunohistochemical and cytogenetic studies indicate that malignant angioendotheliomatosis is a primary intravascular (angiotropic) lymphoma

The authors performed immunohistochemical and cytogenetic studies in a 73‐year old man with malignant angioendotheliomatosis. The patient was referred for evaluation of fever of unknown origin, hepatic failure, and neurologic deterioration. Examination of a muscle biopsy revealed numerous, noncohesive atypical mononuclear cells within small vessels. These cells stained positively with a pan‐leukocyte marker CD45(PD7/26/16) and with a B‐cell marker L26 but negatively with Factor VIII‐related antigen, an endothelial cell marker. Peripheral blood obtained before chemotherapy was cultured and analyzed by the G‐band method. A new translocation and numerous chromosomal aberrations were identified. The major cell line karyotype was 53, XY,+X,+5q?,‐6,+i(6p),+7,‐10,+11,‐12,+12p‐,+12p‐,+18,+marl,+mar2, t(1;3)(p22;p21),3q+,8p+. This is the first cytogenetic study performed in a case of malignant angioendotheliomatosis. Our findings demonstrate that the neoplastic cells in this disorder circulate in the peripheral blood and provide further evidence that malignant angioendotheliomatosis is a diffuse intravascular neoplasm of lymphoid origin. Furthermore, the authors conclude that this malignant lymphoproliferative disorder should be reclassified as a primary intravascular (angiotropic) lymphoma.

The eosinophilic variant of Wegener's granulomatosis

Four cases of an unusual histologic variant of Wegeners granulomatosis, diagnosed by open lung biopsy, are described. In addition to pathergic necrosis, granulomatous inflammation, and vasculitis, intense stromal eosinophilia was observed in the absence of peripheral blood eosinophilia or clinical asthma. A microangiitis was also present in all four cases. Clinically, all patients presented with systemic disease and responded to immunosuppressive therapy including steroids and cyclophosphamide. This form of Wegeners disease poses problems in its distinction from allergic angiitis and granulomatosis, and the differential diagnosis of these two entities is discussed.

Surgical pathology of the lung in anti-basement membrane antibody-associated Goodpasture's syndrome

We report the findings of lung biopsies from five patients with anti-basement membrane antibody-associated Goodpastures syndrome (ABMA-GS). In four patients, pulmonary capillaritis with hemorrhage was found on lung biopsy, confirming that pulmonary capillaritis can be found in ABMA-GS. Although alveolar hemorrhage was the dominant pathologic finding in four of our five patients, all five patients had evidence of injury at the level of the alveolar wall, manifested by hyaline membranes and widening of alveolar walls by edematous connective tissue. In one of our patients, alveolar hemorrhage was only focal, and diffuse alveolar damage was the dominant pathologic finding. This pattern of lung disease has not been previously described in ABMA-GS.

Diffuse arterial narrowing as a result of intimal proliferation: A delayed complication of embolectomy with the Fogarty balloon catheter ☆ ☆☆

Most complications of embolectomy with the Fogarty balloon catheter are recognized early and have received ample attention in the surgical and radiologic literature. However, the delayed complication of diffuse arterial narrowing causing severe ischemia has received little emphasis, perhaps because follow-up arteriography is not always performed. This report describes five patients--women 43 to 62 years of age--with progressive leg ischemia discovered 2 to 4 months after embolectomy with the balloon catheter. Angiography showed a characteristic pattern of severe, smooth narrowing of that portion of the artery in which balloon embolectomy was performed. Pathologic examination of arterial specimens, available in two of the five patients, revealed marked intimal cellular proliferation, which narrowed the arteries severely without evidence of thrombosis, significant atheromatosis, or active arteritis. The cause appears to be intimal damage by the balloon. Embolectomy with the balloon catheter should be done especially carefully in relatively young women.

Histopathology of bacillary angiomatosis of lymph node

Bacillary angiomatosis, a recently characterized pseudoneoplastic vascular proliferation caused by a bacterium identical or related to the cat-scratch disease bacillus, usually presents as cutaneous lesions. We report the histologic findings of this disease involving the lymph nodes of two immunocompromised patients. The lymph nodes showed patchy involvement by coalescent nodules of proliferated blood vessels lined by plump endothelial cells with pale cytoplasm. There were foci exhibiting mild to moderate nuclear atypia. Although neutrophil infiltration was prominent in one case, it was minimal in the other. The interstitium was formed by pink-staining material in hematoxylin-eosin-stained sections, and this proved to be aggregated bacilli on Warthin-Starry stain. Recognition of this potentially fatal disease is important because it is curable with antibiotics.

The diagnosis of Wegener's granulomatosis from transbronchial biopsy specimens

It is widely believed that thoracotomy is necessary to obtain biopsy specimens adequate for the histopathologic demonstration of pulmonary Wegeners granulomatosis (WG). We report five patients with WG who were diagnosed by transbronchial biopsy (TBB). In three cases, a diagnosis of WG was made by TBB alone. In the other two patients, subsequent open lung biopsies confirmed the TBB findings but did not add essential diagnostic information. Our experience suggests TBB may be appropriate as the initial diagnostic procedure in selected cases of suspected WG. This approach requires an understanding of the diverse histologic features of WG and the correlation of clinical and pathologic data.

Phyllodes Type of Atypical Prostatic Hyperplasia: A Report of 3 New Cases

We report 3 new cases of phyllodes type of atypical prostatic hyperplasia. This lesion is characterized by epithelial and stromal proliferation. Stromal changes are the most characteristic finding in phyllodes type of atypical prostatic hyperplasia, which show atypical cells with enlarged, hyperchromatic sarcomatoid nuclei. Mitotic figures are not present. Although the histological appearance may mimic that of cystosarcoma phyllodes of the breast, this pattern is present only focally or not at all in phyllodes type of atypical prostatic hyperplasia. On computerized tomographic imaging phyllodes type of atypical prostatic hyperplasia has a distinct appearance. These patients can be expected to have a benign clinical course and distant metastases have not been reported. Treatment is by surgical excision as in benign prostatic hyperplasia.