You Wen Qian

Indolent T-lymphoblastic proliferation in Castleman lymphadenopathy

Precursor T-lymphoblastic lymphoma (pre-TLL) is a high-grade lymphoma that is invariably fatal if not treated [1]. The presence of precursor T-lymphoblasts outside of the bone marrow and thymus is diagnostic of pre-TLL. Indolent T-lymphoblastic proliferation, a concept introduced in 1999 by Velankar et al. [2], refers to the proliferation of Tlymphoblasts outside bone marrow and thymus without systemic dissemination. There have been three cases reported in the literature, two in the naso/ oropharyngeal region and one that co-existed with a hepatocellular carcinoma [3,4]. Castleman disease was originally described by Castleman et al. in 1956 [5]. The subsequent literature has expanded this disease entity to include at least three distinctive histologic variants: the hyalinevascular, plasma cell and plasmablastic types [6]. Indolent T-lymphoblastic proliferation, to the best of our knowledge, has never been reported in Castleman lymphadenopathy. We report an unusual retroperitoneal mass with a T-lymphoblastic proliferation in a background of Castleman lymphadenopathy, most likely representing indolent T-lymphoblastic proliferation. A 71-year-old man with a 4-year history of rheumatoid arthritis treated with methotrexate and prednisone presented for evaluation of abdominal pain. A computerised tomography (CT) scan of the abdomen with intravenous contrast identified a mass adjacent to the abdominal aorta just proximal to its bifurcation [Figure 1(A)]. The mass had central calcifications and was slightly edematous. A CT-guided percutaneous biopsy of the mass was non-diagnostic. The patient underwent an exploratory laparotomy, and the retroperitoneal mass was resected. During the surgery, distal small bowel changes consistent with Crohn’s disease were also noted. Grossly, the resected specimen contained a 4.5 cm64 cm64 cm encapsulated mass that was surrounded by a rim of tan-yellow fibroadipose tissue. Cut sections revealed a fleshy, tan-pink cut surface with a central area of calcifications and fibrosis measuring 1 cm in greatest dimension. Multiple hemorrhagic areas were present. Microscopically, multiple foci of immature lymphoid cells were identified in the interfollicular region among relatively preserved residual lymphoid follicles [Figure 1(B)]. The extent of these immature foci varied from block to block, occupying about 20– 50% of the total volume. The immature lymphoid cells demonstrated a high nuclear/cytoplasmic ratio, open chromatin and inconspicuous nucleoli. Sheets of plasma cells and proliferating arterioles were also seen in the interfollicular areas, along with mediumsized arteries with thickened walls. No epithelial cells or distinctive spindle cell population was seen. The follicular areas contained hyalinised vessels, some of which radially penetrated involuted germinal centres and concentric layers of the mantle zone. Multiple areas of fibrosis were also noted. Immunohistochemistry demonstrated that the immature lymphoid cells were T-lymphoblasts positive for CD3, CD1a, TdT [Figure 1(C) and 1(D)] with co-expression of CD4/CD8. They were weakly positive for CD10 but negative for CD34. CD21 staining highlighted the follicular mesh in the residual germinal centres, some of which were

Dyserythropoiesis in a child with pyruvate kinase deficiency and coexistent unilateral multicystic dysplastic kidney.

Pyruvate kinase (PK) deficiency is the commonest enzyme deficiency in the glycolytic pathway leading to hemolytic anemia secondary to decreased Adenosine Triphosphate (ATP) synthesis in the red cells. synthesis. PK deficiency due to mutations in the PKLR (1q21) gene leads to highly variable clinical presentation ranging from severe fetal anemia to well compensated anemia in adults. We describe dyserythropoiesis in the bone marrow of a child with transfusion dependent anemia and unilateral multicystic dysplastic kidney (MCDK) mimicking Congenital Dyserythropoietic Anemia type I (CDA type I). Persistently low erythrocyte PK levels and double heterozygous mutations present in the PKLR gene confirmed the diagnosis of PK deficiency. Pediatr Blood Cancer 2014; 61:1463–1465.

A Rare Case of Kikuchi Fujimoto’s Disease with Subsequent Development of Systemic Lupus Erythematosus

Kikuchi Fujimotos disease (KFD) is a rare, immune-mediated, self-limiting disorder with unique histopathological features. KFD is usually seen in young Asian females; however, cases have been reported throughout the world and in all ethnicities. It has been recognized that there is a rare association between Systemic Lupus Erythematosus (SLE) and KFD via sporadic case reports. The exact pathophysiological relationship between these two diseases is still unclear. We report a case of a young Asian female who presented with persistent fever and lymphadenopathy and was diagnosed with Kikuchi Fujimotos disease based on lymph node biopsy; although an SLE workup was done, she did not meet the American Rheumatology Association (ARA) diagnostic criteria for lupus, and the lymph node biopsy did not show features of SLE. She improved clinically with a short course of steroid therapy. Two months later, the patient presented with central facial rash and arthralgia. SLE workup was repeated, a skin biopsy was done, and the results at this time supported a diagnosis of SLE.

A case of peripheral T-cell lymphoma, not otherwise specified in a HCV and HTLV-II-positive patient, diagnosed by abdominal fluid cytology

BACKGROUND Peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) is a rare neoplasm that typically presents as generalized lymphadenopathy. PTCL, NOS presenting as malignant ascites is rare. METHODS A 61-year-old African-American man with past medical history of HCV, cryoglobulinemia, and cryptococcal pneumonia was admitted for dyspnea on exertion over a period of 1 month and new onset of abdominal distension. RESULTS Ascites, splenomegaly, hepatomegaly and extensive lymphadenopathy were found by imaging. Paracentesis obtained 1.3 liter of abdominal fluid, the cytologic evaluation showed a monomorphic population of intermediate-sized lymphoid cells with irregular to convoluted nuclear contours. Fluid sent for flow cytometry showed an abnormal T-lymphocyte population expressing CD4, weak surface CD3 and absence of CD7. PCR studies of ascitic fluid detected a clonal T-lymphocyte population with T-cell receptor gamma gene rearrangement. Serologic testing for human T lymphotropic virus (HTLV) was positive for HTLV-II. Subsequent bone marrow biopsy revealed lymphomatous involvement. CD30 and ALK-1 immunostaining were negative. This case was classified as PTCL, NOS. CONCLUSIONS PTCL, NOS can have unusual clinical presentation such as ascites and pleural effusion, and may also occur as a complication of immunodeficiency state. Further studies are needed to determine if HCV or HTLV-II viral infection is associated with PTCL.

Effect of Freezing Plasma at −20°C for 2 Weeks on Prothrombin Time, Activated Partial Thromboplastin Time, Dilute Russell Viper Venom Time, Activated Protein C Resistance, and d-Dimer Levels

To assess the impact of preanalytical variables of time and temperature on prothrombin time (PT), activated partial thromboplastin time (aPTT), dilute Russell viper venom time (DRVVT), activated protein C resistance (APCR), and d-dimer, samples from 23 healthy individuals and 18 patients having coagulopathy with known abnormal PT and aPTT were collected. Plasma from each individual was separately pooled and aliquoted; the first 2 aliquots were stored at room temperature then analyzed at 2 hours (baseline) and 4 hours postcollection. The remaining aliquots were stored at −20°C and thawed for analysis at 48 hours, 1, and 2 weeks. In both healthy participants and participants with coagulopathy, PT, aPTT, APCR, DRVVT, and D-dimer had no significant changes at 4 and 48 hours, and 1 and 2 weeks postcollection compared to baseline, or the changes were less than 10%. The results indicate PT, aPTT, DRVVT, APCR, and d-dimer can be stored for 2 weeks at −20°C without compromising clinical interpretation in both healthy individuals and individuals with coagulopathy. Increasing storage time will facilitate sample processing from off-site clinics.

A Case of Philadelphia Chromosome Positive Myeloproliferative Neoplasm in a Pregnant Woman with Unusual Primary Myelofibrosis Features

Myeloproliferative neoplasms (MPNs) are traditionally separated into BCR-ABL-positive chronic myeloid leukemia (CML), and BCR-ABL-negative MPNs including primary myelofibrosis (PMF), essential thrombocythemia (ET), and so forth. One of the diagnostic requirements for PMF and ET is the absence of the Philadelphia chromosome, while its presence is almost universally indicative of CML. However, a diagnostic dilemma arises when Philadelphia chromosome-positive MPNs lack the majority of the typical features seen in CML. Some of these classic CML features include basophilIa, marked leukocytosis, neutrophils left-shift with myelocytes bulge, and “dwarf” megakaryocytes. Presented here is a case of a 32-year-old pregnant patient who did not have typical morphologic findings for CML, and yet the Philadelphia chromosome was positive. The patient demonstrated some pathologic features that are commonly presented in PMF that included bone marrow reticulin fibrosis, leukoerythroblastosis, splenomegaly, and increased serum lactate dehydrogenase.

An online decision support system for diagnosing hematologic malignancies by flow cytometry immunophenotyping

An online decision support system for hematopoietic neoplasm based on virtual flow has been developed. Rules were implemented via eXtensible Markup Language (XML). 153 cases representing 28 different hematopoietic neoplasms were correctly classified. Further testing for unknown cases is undergoing.

Sporadic Burkitt Lymphoma Presenting as Acute Pancreatitis, Concurrent Sinusitis, and Enlarged Adenoids

Pancreatitis and sinusitis as presentations of Burkitt lymphoma are uncommon and rarely described in children. We describe here the case of a child who presented with symptoms suggestive of sinusitis unresponsive to antibiotics, with subsequent development of abdominal symptoms due to pancreatitis. He was eventually diagnosed with Burkitt lymphoma.

Steroid-responsive atypical marginal zone hyperplasia of the lip in a child.

Abstract:Atypical marginal zone hyperplasia (AMZH) is a recently described disease entity seen mainly in children. AMZH most commonly affects tonsils and appendices. Cutaneous AMZH is rare. The authors report here a recurrent AMZH in the lip of a 9-year-old child who presented originally with a lip swelling for approximately 3 months. The lip lesion recurred after each incomplete excision for 4 times. Pathologically, the lesion demonstrated marginal zone B-cell hyperplasia with kappa monoclonality by flow cytometry and immunohistochemistry studies. Lymphoepithelial lesions were noted with involvement of minor salivary glands. Polymerase chain reaction for immunoglobulin heavy-chain gene rearrangement has been repeatedly negative. Polymerase chain reaction for Borrelia species DNA was negative on both paraffin-embedded tissue and plasma. Serum antibodies IgG and IgM for Helicobacter Pylori were positive. A diagnosis of AMZH was made. Two courses of anti H. Pylori therapy did not improve the lip lesion, which completely regressed after a course of prednisone therapy. With differential diagnosis of cutaneous marginal zone lymphoma, the case illustrated diagnostic challenges, especially with recurrent lesions. This is the first case of recurrent cutaneous AMZH that has uncharacteristic kappa light-chain restriction. AMZH should be considered in children with mucocutaneous lesions with features of marginal zone lymphoma.

Retrospective Study of Hepatitis C Virus Genotypes and its Association with Lymphoma

Hepatitis C virus (HCV) involves both the liver and extra hepatic organs. The aim of this study was to retrospectively evaluate the association between HCV genotypes and lymphomas. Lymphoma cases were retrieved from our surgical pathology and hematopathology archives from January 2005 to April 2012. Patients who had positive HCV serology with subsequent viral genotyping were selected. Patients with positive Human immunodeficiency virus (HIV) serology were excluded. We identified 17 lymphoma cases with associated HCV infection. Eleven out of 14 (79%) patients had genotype 1 HCV. Diffuse large B cell lymphoma (DLBCL) was the most common lymphoma (6 out of 17 cases) and all cases of DLBCL had genotype 1. Genotype 2 was detected in only three patients (21%) with the diagnoses of follicular lymphoma, splenic marginal zone lymphoma, and classical Hodgkin lymphoma (CHL). CHL was diagnosed in three cases and peripheral T-cell lymphoma in one case.Twelve of 17 (71%) patients were incarcerated in the Texas Department of Criminal Justice system. All 11 genotype 1 patients were male, 4 of 11 (36%) were African American, 4 of 11 (36%) were Caucasian and 3 of 11 (27%) were Hispanic.We concluded that HCV genotype 1 was more common than genotype 2 while no other genotype was detected.