Maria Giovanna Ferrara

Increased hepatitis C viral load and reactivation of liver disease in HCV RNA-positive patients with onco-haematological disease undergoing chemotherapy

AIMS To evaluate changes in Hepatitis C Virus (HCV) RNA both in plasma and Peripheral Blood Mononuclear Cells (PBMC) in onco-haematological patients. PATIENTS AND METHODS 8 consecutive anti-HCV/HCV RNA-positive patients with onco-haematological diseases (5 with B-cell Non-Hodgkin Lymphoma and 3 with chronic lymphocytic leukaemia) were observed during chemotherapy and after its discontinuation. All were naïve to chemotherapy. HCV RNA was sought by Real Time Polymerase Chain Reaction in Light Cycler 1.5 in plasma and PBMC samples collected before, during and after chemotherapy. RESULTS An increase in HCV RNA of at least 1.5 log IU/mL in plasma and 1.1 log IU/ml in PBMC was observed in all 7 patients undergoing Rituximab-based chemotherapy; these patients showed a hepatic flare after discontinuation, life-threatening in one with cirrhosis. Also the 8th patient had cirrhosis, but was treated with Rituximab-sparing chemotherapy and did not show any increase in HCV RNA or a hepatic flare. CONCLUSION Rituximab-based chemotherapy favours an increase in HCV RNA in onco-haematological patients; this is followed by a hepatic flare, possibly immune-mediated and life threatening in cirrhotic patients.

Is it possible to discontinue imatinib mesylate therapy in Chronic Myeloid Leukemia patients with undetectable BCR/ABL? A case report and a review of the literature.

Imatinib mesylate (IM) therapy leads to a complete cytogenetic response (CCyR) in 75-90% of Chronic Myeloid Leukemia (CML) patients in chronic phase, but only a small percentage of patients achieve complete molecular response (CMR). Very little is known about IM discontinuation. We report the case of a 20-years-old male patient in chronic phase CML who maintained undetectable BCR/ABL mRNA levels, despite IM discontinuation over a period of 15 months after achieving CMR. Our patient reached CCyR and CMR after 3 and 6 months of IM treatment, respectively. We also reviewed the published literature concerning cases of IM discontinuation.

Serum double monoclonal components and hematological malignancies: Only a casual association? Review of 34 cases

A double monoclonal component (MC) detected in the serum and/or urine represents a very rare occurrence (2-6% of monoclonal gammopathies). In this study, we report 34 patients with double serum MCs, focusing on the associated diseases. The diagnosis was made using high-resolution serum protein electrophoresis and immunofixation. Of the 1214 patients with monoclonal gammopathies, 49 had a double MC but only 34 (2.8%) were included in our study. A double MC was associated with hematological malignancies in 20/34 cases. Based on our experience, a double MC is more often associated with other diseases, especially an oncohematological one.

Clinical significance of serum triple monoclonal components: a report of 6 cases and a review of the literature.

A serum multiple monoclonal component (MC) is very rare. We here report 6 patients with 3 MCs. The triple MC was detected in all of them by immunofixation. 2/6 patients did not present hematological or oncological associated disease, while in the remaining 4, Waldenström macroglobulinaemia (2 cases), Polycythemia Vera and non-Hodgkin lymphoma were diagnosed. Of the 49 global patients reported in the literature (6+43), 64.6% had a lymphoproliferative disorder and only in 3 cases there was no associated disease. Therefore, the detection of such laboratory evidence should propel physicians to a deeper investigation.

Bilateral Lacrimal Gland Involvement With Mantle Cell Lymphoma

A 73-year-old woman presented with a rapidly growing bilateral orbital swelling, causing diplopia and epiphora. During physical examination, there were exophthalmos and orbital edema, both more prominent in the right eye (Figures 1A and 1B). The conjunctiva of both eyes was swollen. The lesions extended to the right iris and partially covered the left one. Computed tomography revealed, in both eyes, a mass (right eye: 2.7 cm 3 2.14 cm; left eye: 1.88 cm 3 1 cm) involving the lacrimal gland and adjacent to the superior rectus and lateral rectus muscles. Histological and immunohistochemical analyses showed a population of small atypical lymphocytes, with slightly irregular indented nuclei and moderately dispersed chromatin. Malignant cells were CD19+, CD20+, CD5+, CD232, CD102, and hyperexpression of cyclin D1 was detected. Cytogenetic analysis demonstrated the presence of a complex karyotype: t(11; 14)(q13; q32), t(7; 10)(q21; p12), dup (11)(q25, q13), and 217. The final diagnosis was mantle cell lymphoma (MCL), stage IV-A. The patient underwent 6 cycles of R-GIFOX (Rituximab-Gemcitabine, Ifosfamide, Oxaliplatin) achieving a complete response. Unfortunately, 18 months later, her disease relapsed (although at a different site), and she died 29 months after the initial diagnosis. Ocular adnexal lymphoma (OAL) accounts for approximately 1% to 2% of all non–Hodkgin’s lymphomas (NHL). OAL comprises both primary extranodal NHL and secondary involvement in systemic lymphomas. Although mucosa-associated lymphoid tissue represents the most common histology, MCL is observed in only very small percentages. MCL represents between 2% to 10% of all NHLs, and it has often an aggressive clinical behavior. The chromosomal translocation t(11; 14)(q13, q32), that juxtaposes the cyclin D1 and the immunoglobulin heavy chain genes, is detected in almost all cases, and it represents the molecular hallmark of MCL. The aim of treatment for younger patients still remains a long-lasting complete remission. In elderly patients treatment still represents a challenge. For fit elderly patients, long progression-free survival and molecular remissions are possible, whereas for the frail patients, the treatment should aim at reducing symptoms and maintaining quality of life (3).