ALBERT

Description: Background: CD is a rare entity characterized by unicentric or multicentric clinical presentation. Three histologic variants (hyline vascular, plasmacell and mixed) have been described. Coexistence of CD and non-Hodgkin (NHL) and Hodgkin’s (HL) lymphomas is well documented in literature; however the last type of association is a rare event. When it occurs, the most frequent association is between interfollicular subtype of HL and plasmacell variant of CD, while there is no agreement regarding a more common combination of HL and either the multicentric or the localized form of CD. Methods and Results: On July 2000, a 33-years-old woman HIV-negative was found to be affected by NS grade I of BNLI cHL diagnosed on a supraclavear nodal biopsy. The final stage was IIIA. After 6 cycles of ABVD and mediastinal radiotherapy, the patient obtained a CR (January 2001). One year later (May 2002) she complained asthenia, but not other clinical signs or symptoms. Laboratory tests revelead increased ERS (49 mm/h) and only mild anemia (11,7 mg/dl). Computerized tomography (CT), ultrasonography (US) and positron emission tomography (PET) showed multiple subdiaphgramatic enlarged lymphnodes in celiac tripod region, haepatoduodenal ligament and interaortocaval zone (2–3 cm of diameter). However, the biopsied nodes obtained by laparoscopy reveled a mixed reactive lymphoadenopathy, but not recurrent HL. During the following 13 months of observation, a further reduction in the hemoglobine value (10.9 mg/dl), increased ERS (87 mm/h) and a very slowly progressive increase in the dimension (4–5 cm diameter) of the abdominal enlarged nodes were documented in the absence of others clinical signs or symptoms. A second nodal biopsy (paracaval lymphnode) was performed in October 2003. Histology showed some lymphoid follicles with small germinal centers(GC), which presented prominent hyalinized vessels and politypical plasmacell component in interfollicular areas indicative of CD (mixed hyaline-vascular and plasmacell subtype). Rare Reed-Stenberg and lacunar cells were found in this background. After the second course of chemoterapy (6 cycles of ABVD) a rapid normalization of ERS and anemia were documented. The CT findings showed a reduction of size and numbers, but no disappereance of abdominal lymph nodes (4 residual nodes of 1–2 cm of diameter). A further nodal biopsy was performed, which confirmed the persistence of HL in the background of CD. Only after RT, the patient obtained a second CR as documented by two subsequent negative CT and PET scans. On July 2005, even if laboratory tests were normal, a single ipogastric superficial lymphadenopathy was detected on phisical examination and confirmed by US: so, a new diagnostic work-up, consisting of PET-SCAN and escixional biopsy is ongoing. Conclusion: After cHL sclero-nodular type, the diagnosis of recurrent HL probably arising on a preexisting multifocal CD, both caracterized by a particularly indolent course, was documented. The two pathogenetic mechanisms hypothized for CD and HL association are: secretion of IL-6 by Hodgkin’s RS cells and/or histiocytes or manifestation of an abnormal immune state associated with Hodgkin’s disease.

Description: BACKGROUND Extramedullary blast crisis (BC) of Ph’+ CML is infrequent and commonly affects bone, lymphoid tissue, skin and soft tissues and central or peripheral nervous system. Most of nodal lymphoid tumors occuring in the setting of CML derive from T-cell precursors and represent the evolution of CML to a lymphoid nodal BC. CASE HISTORY In February 2004, a 52 year-old patient underwent diagnostic wide biopsy of a nasopharingeal mass that caused severe acute respiratory symptoms. At the histological examination, the nasopharyngeal mucosa exhibited a diffuse pattern of infiltration by neoplastic cells with a characteristic single-file arrangement. The cells showed typical convoluted nuclei with one or two nucleoli and abundant cytoplasm (L2, lymphoblasts). The malignant cell population expressed a preT-cell immunophenotype: cytoplasmic CD3(+), CD43 (+), TdT (+/−), CD34(+), CD4(−), CD8(−). Thus, the diagnosis of T-cell LL was formulated. Whole-body CT scan revealed nasopharingeal mass, retropharyngeal, laterocervical, axillary, inguinal enlarged nodes and splenomegaly. Laboratory tests indicated leukocytosis (58,000/μl) with a differential WBC count typical of CML in chronic phase (myeloblasts