Primary renal lymphoma (PRL) is a rare form of extranodal non-Hodgkin's lymphoma often diagnosed and treated by oncologists and urologists. Pathophysiological and clinical data on PRL are sparse, but the limited reported experience suggests the disease usually has an ominous outcome. As in other renal tumors, comprehensive radiological investigations have a central role in the recognition and final diagnosis of PRL. We describe the presenting features and clinical course of an elderly woman who was found to have PRL after evaluation for persistent low-grade fever. Diagnostic and therapeutic caveats are discussed on the basis of a critical literature review of case reports and descriptions of small series of patients.

Although primary effusion lymphoma (PEL) is usually associated with human herpes virus-8/Kaposi sarcoma herpes virus (HHV-8/KSHV) and human immunodeficiency virus (HIV), there are several reports of HHV-8/KSHV and HIV negative cases, mainly in the setting of immunodeficiency. Here, we report the second case of PEL associated with idiopathic T4 lymphocytopenia (ICL), which was HHV-8/KSHV negative, HIV negative and Epstein-Barr virus positive, while no other causative agents for immunodeficiency were documented. Flow cytometry revealed a hyperdiploid and highly mitotic large B-cell population, CD30, EMA, CD66, CD38 and CD71 positive. The malignant lymphoma cells showed atypia with prominent nuclei and basophilic vacuolated cytoplasm, while cytogenetic analysis with fluorescent in situ hybridization showed trisomy 18. The patient was administered R-COP chemotherapy, but no remission was achieved, up to 3 months from diagnosis.

Objective: HYPER-CVAD is an intensive treatment protocol of short duration, for ALL and other lymphoid neoplasms. It is consisted of 8 alternating cycles (parts A and B): in part A (cycles 1, 3, 5, 7) fractionated doses of cyclophosphamide, vincristine, doxorubicin and dexamethazone are administrated, while in part B (cycles 2, 4, 6, 8) high doses of methotrexate and aracytin. The two first cycles compose the induction therapy, while the next 6 cycles constitute the consolidation therapy, followed by two years of maintenance therapy. Protection of CNS is achieved with intradorsal injections, whereas in Ph+ ALL patients, imatinib is also administered. The aim of this study was the clinical evaluation of MRD detection in adult patients with ALL, during chemotherapy with HYPER-CVAD. Patients/Methods: During the period 1999–2008, 30 patients were hospitalized in our hospital for ALL and were treated with HYPER-CVAD therapeutic protocol. Among them, 14/30 (46,7%) were males and 16/30 (53,3%) females (median age 43,5 years, range 16–70). Median follow-up time was 12,8 months (range 0,5–100). ALL of T-origin had 8/30 patients and of B-origin, 22/30 (1 B1-EGIL/pro-B, 17 B2-EGIL/B-common, 4 B3-EGIL/pre-B). Caryotypic analysis and FISH was done in all patients (7/30 bcr/abl+). According to classical prognostic markers of ALL: 21/30 were classified as high, 4/30 as medium and 5/30 low risk, respectively. MRD presence was detected in bone marrow samples, with flow cytometric panels, at three particular treatment time-points: completion of induction therapy (T1), completion of consolidation therapy (T2) and at the end of maintenance therapy (T3). Overall survival (OS) and disease free survival (DFS) were investigated, especially in relation to the influence of MRD presence in (OS) and (DFS), respectively. For statistical analysis, Kaplan-Meier was used. Results: At treatment time-point (T1), MRD was detected in 10/28 (35,7%) patients, at time-point (T2) in 7/23 (30,4%) and at treatment-point (T3) in 9/17 (52,94%). The influence of MRD detection in (OS) was statistically significant (p<0,05) at (T2) and (T3), while in (DFS) the detection of MRD presence in any of the standardized treatment time-points resulted in decreased DFS (T1/p<0,05, T2 and T3/p<0,001). Conclusions: Our results suggest that MRD detection during therapy of adult ALL is a negative prognostic indicator for (OS) and (DFS), the independency of which has to be confirmed after prolonged follow-up time and increased number of cases.

Aggressive lymphomas can present with symptoms mimicking life-threatening infection. Flow cytometry (FC) is usually recommended for the classification and staging of lymphomas in patients with organomegaly and atypical cells in effusions and blood, after the exclusion of other possible diagnoses. FC may also have a place in the initial diagnostic investigation of aggressive lymphoma. Three cases are presented here of highly aggressive lymphomas in young adults, which presented with the clinical picture of fever of unknown origin (FUO) in patients severely ill. All followed a life-threatening clinical course, and two developed the hemophagocytic syndrome (HPS), but microbiological, immunological, and morphological evaluation and immunohistochemistry (IHC) failed to substantiate an early diagnosis. FC was the technique that provided conclusive diagnostic evidence of lymphoma, subsequently verified by IHC. Our experience with these three cases highlights the potential role of FC as an adjunct methodology in the initial assessment of possible highly aggressive lymphoma presenting with the signs and symptoms of life-threatening infection, although the definitive diagnosis should be established by biopsy. In such cases, FC can contribute to the diagnosis of lymphoma, independently of the presence of HPS.

Background There are no immunophenotypic guidelines for the investigation of MYC‐rearranged lymphomas. We aimed to identify simple immunophenotypic features that would help to differentiate between MYC‐rearranged lymphomas and guide cytogenetic analysis. Methods We reviewed diagnostic samples from patients diagnosed with Burkitt lymphoma (BL), double‐hit lymphoma (DHL), MYC‐rearranged diffuse large B‐cell lymphoma (MYC‐DLBCL), and standard (non‐MYC‐rearranged) DLBCL over the last decade in our Institution. Using flow cytometry (with antibodies CD20, CD10, CD38, bcl‐2, Ki‐67, FMC‐7, CD43, CD27, CD79b, CD23, and CD22) we determined antigen% expression and median‐fluorescence intensity ratios (MFIR). The forward scatter (FS) and side scatter (SS) characteristics of tumor B‐cells were compared with normal T‐cells (B/T ratios) for patients with MYC‐rearranged lymphomas. Results We identified 51 patients of whom 14 had BL, 10 had DHL (6 MYC+/BCL2+; 4 MYC+/BCL6+), 8 MYC‐DLBCL, and 19 standard DLBCL. The significant differences (p <.05) were: higher CD38% in BL than standard DLBCL; higher CD10% in BL and DHL versus MYC‐DLBCL and standard DLBCL; higher CD10MFIR in BL than MYC‐DLBCL and standard DLBCL; higher Ki‐67% in BL than DHL and MYC‐DLBCL; higher bcl‐2% in DHL than BL; higher FMC‐7% in BL than MYC‐DLBCL and standard DLBCL; and lower SS (B/T) ratio in DHL than MYC‐DLBCL. Conclusions The combination of CD38% > 90, CD10% > 80, CD10MFIR > 10, bcl‐2% < 30, and Ki‐67% > 70 was characteristic of BL. “Deviation” from these cut‐offs should raise suspicion for DHL and, therefore, BCL2 and/or BCL6 FISH is required. We also found that a diagnosis of DHL rather than of MYC‐DLBCL was significantly associated with CD10% > 60, Ki‐67% > 50, and SS (B/T) <1.5.