Case study documenting the diagnosis of idiopathic CD4+ Lymphocytopenia in a patient with atypical fungal infection (disseminated blastomycosis) by FNA of adrenal mass.

Idiopathic CD4+ lymphocytopenia, described in 1992 by the Centers for Disease Control, is characterized by persistent CD4+ lymphocytopenia (less than 300 cells per micro-liter) in nonimmunosuppressed, HIV negative individuals, who present with atypical infections. This rare though likely undiagnosed entity is associated with chronic disseminated forms of either fungal or bacterial infections in otherwise healthy adults. We report a case of a 59-year-old male with ring-enhancing brain lesions, bilateral adrenal masses, lung and vocal cord nodules, where the diagnosis of exclusion was metastatic malignancy. Fine needle aspiration (FNA) of the adrenal mass and a subsequent vocal cord biopsy confirmed chronic widely disseminated blastomycosis. Flow cytometric evaluation of peripheral blood documented persistent selective CD4+ lymphocytopenia with T8 (suppressor) T-Lymphocyte count within normal range. We believe that idiopathic CD4+ lymphocytopenia is an important etiologic factor to be considered for patients who present with mass lesions and are diagnosed by FNA with atypical fungal infections. We relate the diagnostic criteria for idiopathic CD4+ lymphocytopenia and the importance of providing on-site triage for FNA samples for fungal studies and correlation for flow cytometry.

Retroperitoneal cystic abdominal lymphangiomatosis diagnosed by fine needle aspiration: a case report.

BACKGROUND: Attributed to congenital malformation of lymphatic ducts, diffuse retroperitoneal cystic abdominal lymphangiomatosis has a distribution that often corresponds to the location of primitive fetal lymphatic sacs. Three recognized types are capillary, cavernous and cystic. Multisystem involvement may occur involving spleen, liver, bone, pancreas, soft tissue, limbs and brain. CASE: A 55-year-old, healthy male with multiple liver lesions and retroperitoneal lymphadenopathy presented for retroperitoneal fine needle aspiration, producing 20 mL of milky liquid. Immediate cytologic evaluation showed a heterologous population of mature lymphocytes with chylomicrons. Flow cytometry revealed a polyclonal population of mature lymphocytes. Chemical analysis demonstrated a normal serum cholesterol level and an elevated triglyceride level. Serum markers were noncontributory. We review the differential diagnostic considerations leading to obstruction or retention of lymphatic fluids (malignancy, surgical, infective and traumatic), with an emphasis on the importance of onsite cytologic evaluation, correlation with clinical history and review of the etiologic considerations. CONCLUSION: The constellation of clinical, radiologic, cytologic and laboratory findings presented in this paper are diagnostic of diffuse retroperitoneal cystic abdominal lynmphangiomatosis. To our knowledge, this entity has not been reported before in the fine needle aspiration literature. To prevent further disruption of lymphatic drainage, no further surgical sampling is planned.

Giant cell arteritis presenting as facial swelling.

Facial swelling is commonly ascribed to angioedema and a host of other causes. Temporal arteritis (TA), a disease most often diagnosed in patients over the age of 50 years, frequently presents with nonspecific and often ignored complaints (headache, symptoms of polymyalgia rheumatica, low-grade fever, fever of unknown origin, loss of appetite, depression, joint pains, weight loss, hair loss, and even respiratory symptoms). The diagnosis of TA is highly likely in the presence of new-onset headaches, polymyalgia rheumatica, and a tender, cord-like, or swollen temporal artery. Facial swelling must be appreciated as another presentation of TA, especially when accompanied by other nonspecific symptoms. High clinical suspicion, immediate treatment, and definitive diagnosis by temporal artery biopsy are necessary to prevent the most severe vascular complications of blindness and cerebrovascular accidents. Treatment with corticosteroids is most often successful. Because this treatment is fraught with all the risks of high-dose and prolonged steroid therapy, it should only be initiated in cases of significant clinical suspicion, followed by a timely temporal artery biopsy to confirm the diagnosis. Delay in therapy increases the risk of a vascular catastrophe. Delay in obtaining a temporal artery biopsy after therapy has been initiated decreases the diagnostic sensitivity of the test. Other modalities of immunosuppressive therapy remain either unsuccessful or unproven. Concomitant low-dose aspirin therapy appears to hold promise.

T-cell lymphoproliferative disorder of hand-mirror cell morphology presenting in an eosinophilic loculated peritoneal effusion, with omental "caking".

BACKGROUND: Cells with "hand mirror" morphology have not, to the best of our knowledge, been described in a primary effusion sample. This paper describes a case of T-cell lymphoma with eosinophilia in a patient with suspected peritoneal carcinomatosis. Rarely, a T-cell lymphoproliferative process may mimic primary peritoneal carcinomatosis, clinically suggested by a presentation in CT imaging of omental caking with bilateral massive loculated effusions in a patient without lymphadenopathy or splenomegaly. METHODS: A 60 year old caucasian male presented with vague abdominal discomfort and increasing abdominal girth. Computed tomography showed a two centimeter thick omental cake and a small loculated effusion. The clinical presentation and imaging findings were most consistent with peritoneal carcinomatosis. Cytologic evaluation of the effusion was undertaken for diagnostic study. RESULTS: Rapid intraprocedural interpretation of the effusion sample showed a monomorphic population of cells with "hand-mirror" cell morphology exhibiting cytoplasmic extensions (uropodia) with 3-5 course dark cytoplasmic granules and a rim of vacuolated cytoplasm capping the opposing "mirror head" side. These cells were seen within a background of mature eosinophils. Flow cytometric evaluation of the ascites fluid demonstrated an atypical T-cell population with the following immunophenotype: CD2-, CD3+, CD4-, CD5-, CD7-, CD8+, CD56+. T-cell receptor (TCR) gene rearrangement was positive for clonal TCR-gamma gene rearrangement, supporting the diagnosis of a T-lymphoproliferative disorder. CONCLUSION: A T-cell lymphoproliferative process may present with "hand mirror" morphology in an effusion sample. These cells may show polar cytoplasmic vacuolization and 3-5 course granules within the "handle" of these unique cells. Cytoplasm shows peripheral constriction around the nucleus.

Sporadic ganglioneuromatosis of esophagogastric junction in a patient with gastro-esophageal reflux disorder and intestinal metaplasia.

A 58-year-old female with a recurrent history of upper abdominal pain and intermittent dysphagia underwent endoscopic evaluation that demonstrated an irregular and nodular esophago-gastric (EG) junction and grade I erosive esophagitis. Biopsies showed prominent intestinal metaplasia of Barrett's type without dysplasia, chronic inflammation and multiple aggregates of large cells within the mucosal lamina propria, some with spindle shaped nuclei. Immunohistochemistry stains for keratins AE-1/AE-3 were negative, while S-100 and NSE were positive. This, together with routine stains, was diagnostic for mucosal ganglioneuromatosis. The background of chronic inflammation with intestinal type metaplasia was consistent with long-term reflux esophagitis. No evidence of achalasia was seen. Biopsies of gastric antrum and fundus were unremarkable, without ganglioneural proliferation. Colonoscopy was unremarkable. No genetic syndromes were identified in the patient including familial adenomatous polyposis and multiple endocrine neoplasia type IIb (MEN IIb). Iansoprazole (Prevacid) was started by oral administration each day with partial relief of symptoms. Subsequent esophagogastroscopy repeated at 4 mo showed normal appearing EG junction. Esophageal manometry revealed a mild non-specific lower esophageal motility disorder. Mild motor dysfunction is seen with gastro-esophageal reflux disease (GERD) and we feel that the demonstration of localized ganglioneuromatosis was not likely related etiologically. In the absence of findings that might suggest neural hypertrophy, such as achalasia, the nodular mucosal irregularity seen with this instance of ganglioneuromatosis may, however, have exacerbated the patient's reflux.

Subperitoneal placenta accreta succenturiate in the case of a successful near-term extrauterine abdominal pregnancy.

Placenta from an extrauterine abdominal pregnancy was examined after a 37-week healthy infant gestation. The placenta, with its fetal surface down and maternal surface up, protruded from the pelvic area to peritoneal cavity in the wall of the amniotic sac containing fetus. The placenta was implanted under the thin subperitoneal layer of maternal tissue completely covered by peritoneal serosa and was formed by several small lobes connected by intramembranous placental vessels. Insertion of the trivascular umbilical cord was velamentous. Partially remodeled arteries infiltrated by intermediate trophoblast and frequent veins directly communicating with the placental intervillous space were identified in the subperitoneal maternal tissue. The term "placenta accreta" is appropriate in this case because villi in the basal plate implanted directly in the maternal subserosal connective tissue without intervening decidua.

Cutaneous anthrax: conservative or surgical treatment?

This article summarizes the diagnostic features and treatment recommendations for cutaneous anthrax, exemplified by a case report of nontypical cutaneous anthrax. The treatment of choice is medical, with ciprofloxacin or doxycycline the preferred antibiotics. However, surgical biopsy may be used if the clinical setting and microbiologic examination of swabs are not diagnostically conclusive. Histopathologic findings explain the clinical observation that most cutaneous anthrax lesions heal without scar formation.

Prevalence of presumptive Bacillus anthracis in the human population examined by nasal swabs.

Routine cultures may reveal presumptive Bacillus anthracis microorganisms in human samples. Our study of 1336 individuals showed the probability of encountering presumptively positive cultures using routine microbiologic examination was approximately 0.4% (5 individuals) when nasal swabs were examined for the following characteristics indicative of Bacillus anthracis : nonhemolytic ground-glass colonies retaining their shape when manipulated, nonmotile in testing media, and microscopically revealing spore-forming gram-positive rods. Confirmatory tests revealed that those cultures were of Bacillus nonanthracis microorganisms. The probability appears significantly high to suggest a need for the confirmatory tests to be available at the community laboratories. In addition, our study confirmed the Gram's staining method does not kill all the spores of the spore-forming gram-positive rods, necessitating special safety requirements.