γδ T-Cell Acute Lymphoblastic Leukemia/Lymphoma: Discussion of Two Pediatric Cases and Its Distinction from Other Mature γδ T-Cell Malignancies.

Gamma delta (γδ) T-cell antigen receptor (TCR) expression and its related T-cell differentiation are not commonly reported in T-cell acute lymphoblastic leukemia/lymphoma (T-ALL). Here we report two pediatric T-ALL cases and present their clinical features, histology, immunophenotypes, cytogenetics, and molecular diagnostic findings. The first patient is a two-year-old girl with leukocytosis, circulating lymphoblasts, and a cryptic insertion of a short-arm segment at 10p12 into the long-arm segment of 11q23 resulting in an MLL and AF10 fusion transcript, which may be the first reported in γδ T-ALL. She responded to the chemotherapy protocol poorly and had persistent diseases. Following an allogeneic bone marrow transplant, she went into remission. The second patient is an eleven-year-old boy with a normal white cell count, circulating blasts, and a normal karyotype, but without any immature cellular markers by flow cytometric analysis. He responded to the chemotherapy well and achieved a complete remission. These cases demonstrate the diverse phenotypic, cytogenetic, and molecular aspects of γδ T-ALL. Early T-precursor- (ETP-) ALL and their differential diagnosis from other mature γδ T-cell leukemia/lymphomas are also discussed.

Nicotinamide Phosphoribosyl Transferase a Reliable Marker of Progression in Cervical Dysplasia.

BACKGROUND/AIM: Nicotinamide phosphoribosyl transferase (Nampt) catalyses the rate-limiting step of the mammalian nicotinamide adenine dinucleotide (NAD) salvage pathway. Nampt is highly expressed in several epithelial and mesenchymal neoplasms, where is promotes cell-cycle progression ans chemotherapy resistance. To our knowledge, alterations in Nampt expression have not been examined in cervical intraepithelial neoplasia (CIN) or squamous cell carcinoma (SCC). MATERIALS AND METHODS: We performed immunohistochemical analysis for Nampt using tissue microarrays on 14 samples of benign cervical squamous epithelium and 15 CIN I, 15 CIN II, and 13 samples of CIN III. The SCCs included 5 low-grade, 67 intermediate-grade, and 81 high-grade tumors. RESULTS: Nampt levels increased with increased CIN grades were compared to benign cervical squamous epithelium. Similarly, Nampt levels increased with increasing SCC grade. CONCLUSION: Nampt expression is a reliable marker of progression in cervical dysplasia and SCC.

ABSOLUTE PLATELET REFRACTORINESS ASSOCIATED WITH HLA ANTIBODIES: A CASE REPORT.

Refractoriness to platelet transfusion is a complex process that can be due to a diverse array of etiologies. We report a case of refractoriness in a patient with acute myelogenous leukemia (AML) and the diagnostic challenge associated with it. During the course of myeloablative therapy the patient demonstrated no response to multiple sequential platelet transfusions given to prevent the onset of bleeding complications in the setting of severe thrombocytopenia. Diagnostic evaluation revealed multiple potential underlying etiologies and contributing factors, with alloimmunity to HLA antigens determined to be the most probable cause after thorough laboratory investigation.

ATYPICAL HEMOLYTIC UREMIC SYNDROME IN AN ADULT SUCCESSFULLY TREATED WITH ECULIZUMAB.

Atypical Hemolytic Uremic Syndrome is a triad of microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure not associated with diarrhea. It is a rare condition associated with complement disorders in about 50 percent of cases. The first line of treatment is therapeutic plasma exchange. However, because clinical response to TPE varies, an anti-complement drug, eculizumab has been tried. We report a case of atypical HUS successfully treated with eculizumab.

Maximizing the diagnostic yield from bone marrow aspirate material using the cell block technique on clot sections.

BACKGROUND: Paraffin section of bone-marrow aspirate (clot section) is one of several components of bone marrow biopsy. Improper acquisition of aspirate material results in lack of diagnostic tissue and a waste of resources. OBJECTIVE: To detail a novel cell block method of aspirated marrow as a way to ensure maximum yield. This is of particular value when the material gathered via core biopsy is inadequate. METHOD: We used the cell block method to evaluate paraffin-embedded sections of hematopoietic tissue from bone marrow aspirate. RESULTS: The range of diagnoses possible from an adequate clot section parallels those from a comparable core biopsy. Examples of an adequate clot section include lesions assessed by routine hematoxylin-eosin (H&E) staining, special and immunohistochemistry (IHC) stains, and molecular diagnostic studies such as fluorescent in situ hybridization (FISH) and polymerase chain reaction (PCR). CONCLUSION: A bone marrow clot section comprised of sinusoidal blood is inadequate for morphological interpretation and is a waste of resources. Hence, we recommend the cell block technique for procurement; this method ensures maximum capture of material needed to establish a diagnosis.

Bisphosphonate-related osteonecrosis of the jaw.

Although there has been a growing body of literature about bisphosphonates since 1969, it was not until 2003 that treatment with this medication was associated with osteonecrosis of the jaw. Presented herein is such a case.

Oligosecretory biclonal multiple myeloma.

Among the plasma cell dyscrasias, non-secretory myeloma is one of the rarest. This diagnosis is based on the absence of monoclonal proteins in the serum and urine. When serum free light chains are trace and the kappa: lambda ratio normal, clonality may however be established by PCR. We present a case of an oligosecretory myeloma confirmed by PCR, which would have hitherto been classified as non-secretory.

Large cell lymphoma associated with prosthetic joint debris.

Soft tissue reactions to materials in joint prostheses include discoloration, fibrosis, florid histiocytic reaction, and granulomatous inflammation with foreign body giant cell reaction. Clinical manifestations include pain and swelling. We report a case of temporomandibular joint Proplast-Teflon prosthesis, followed by the development of large cell lymphoma in the left parotid gland 10 years after joint replacement. While it is unclear whether the implant directly contributed to the development of lymphoma, this association has not been previously documented, prompting this report.

Is platelet transfusion necessary prior to a central venous catheter placement in thrombotic thrombocytopenic purpura patients?

Due to concern for bleeding from severe thrombocytopenia, some thrombotic thrombocytopenic purpura (TTP) patients receive platelet transfusion prior to central venous catheter placement. However, studies have shown that blood loss associated with this procedure is minimal, and platelet administration is unnecessary. In our study, 11 patients with TTP were identified. Before central line placement, two of the 11 received platelet transfusions, while nine did not. Blood loss in all patients was not significant, supporting the position that platelet transfusion prior to central venous catheter placement in TTP patients is unnecessary.

Karyopyknotic cytoplasmic inclusions in neutrophils.

Karyopyknotic cytoplasmic inclusions in neutrophils (KPCI) have been previously called "Howell-Jolly" like inclusions and identified in immunosuppressed patients with human immunodeficiency virus (HIV) and in patients with various malignancies who have undergone chemotherapy. Attempts to characterize these inclusions have included the Grocott's methenamine silver (GMS), periodic acid Schiff (PAS), Gram, and Feulgen stains. Previous authors have concluded that these inclusions are of deoxyribonucleic acid (DNA) origin. We present a case describing an additional method to confirm this observation and to document previously undescribed curvilinear forms.

Fine needle aspiration biopsy of monophasic spindle synovial sarcoma of lung with fluorescence in situ hybridization identification of t(x;18) translocation: a case report.

BACKGROUND: Primary monophasic spindle synovial sarcoma can occur in areas with no apparent relation to synovial structures. The diagnosis can be challenging because of the ability to mimic other spindle cell neoplasms. Within the lung, these neoplasms are rare and cytologic descriptions are limited. CASE: A 58-year-old woman was diagnosed with colonic adenocarcinoma; chest computed tomography (CT) revealed a 5-cm solitary pulmonary mass, and CT-guided fine needle aspiration was performed. Aspirate smears were cellular, with large, loosely cohesive complex tissue fragments that showed dense spindled cells with numerous single stripped spindle cells. Spindle cells were bland and monomorphic, with minimal cellular variation. There was no anaplasia or specific mesenchymal differentiation. Immunohistochemical stains on the cell block were positive for vimentin and bcl-2. A diagnosis of spindle cell neoplasm was rendered; it was believed to be a second neoplasm unrelated to the colonic adenocarcinoma. The main diagnostic consideration was synovial sarcoma. On resection, the neoplasm demonstrated t(x:1 8) chromosomal translocation by fluorescence in situ hybridization. CONCLUSION: In a spindled cell neoplasm arising as a single peripheral pulmonary nodule, monophacir spindle synorvial sarcoma should be considered in the differential diagnosis; detection of the t(x;18) chromosomal translocation can confirm the diagnosis.