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.

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.