Anne Kornreich

Subcutaneous panniculitis-like T-cell lymphoma: further evidence for a distinct neoplasm originating from large granular lymphocytes of T/NK phenotype

We report the case of a 20 year‐old caucasian woman who presented a primary subcutaneous panniculitis‐like T‐cell lymphoma (SPTCL) as an invasive tumor of the chest wall. Herein, the neoplastic cells were found to express a CD3+CD8+ phenotype but also displayed variably the natural killer (NK)‐associated antigens CD56 and CD57 as well as granzyme B. On cytological examination, these cells showed a large granular lymphocyte (LGL)‐like morphology with presence of azurophilic granules in their cytoplasm. Electron dense and membrane bound granules like those found in cytotoxic T lymphocytes (CTL) were also demonstrated by electron microscopy. Neither rearrangement of the T‐cell receptor subunits nor Epstein‐Barr virus (EBV) genome was observed at the molecular level. The LGL‐like features of the neoplastic cells found in this case and the presence of NK‐associated antigens provide additional support to the cytotoxic derivation of most SPTCL.

Morphologic and phenotypic changes of the leukemic cells in a case of marginal zone B-cell lymphoma

Abstract A particular case of marginal zone B-cell lymphoma (MZBCL) presenting with leukemic lymphocytes is reported. In the present observation, the leukemic cells not only displayed a remarkable morphological fluctuation but also had an unusual phenotype, changing with time. These phenotypic features, which have been functionally investigated by in vitro assays, might simply reflect an activation state depending on the microenvironment. Because of its disconcerting similarities with hairy cell leukemia (HCL) and splenic lymphoma with villous lymphocytes (SLVL), this case relaunches the debate about whether close relationships might exist between the splenic marginal zone, SLVL and HCL.

Nature of the T Lymphocytes in Lymphocyte Predominance Hodgkin's Disease

Little is known about the function of the T lymphocytes in lymphocyte predominance Hodgkins disease. We report here the case of a 37-year-old man with a diffuse LPHD, featuring a similar increase in T lymphocytes in both the peripheral blood and the tumor, thus allowing for their characterization by functional assays. These cells were CD4+CD45RO+ and produced high amounts of IL-2 and IFN-gamma, consistent with a TH1-type profile. This subset of T helper cells is involved in cellular immunity and could reflect a cytotoxic reaction directed against the neoplastic cells.

Lack of responsiveness to 1-desamino-d arginin vasopressin (desmopressin) in male patients with nephrogenic syndrome of inappropriate antidiuresis: from bench to bedside

Eur J Clin Invest 2012; 42 (3): 254–259

TH1‐TYPE IMMUNE RESPONSE IN LYMPHOCYTE‐PREDOMINANCE HODGKIN'S DISEASE

Simmons, R.L. & Brunning, R.D. (1979) Virus-associated hemophagocytic syndrome: a benign histiocytic proliferation distinct from malignant histiocytosis. Cancer, 44, 993-1002. Shirono. K. 81 Tsuda. H. (1995) Parvovirus B19-associated haemophagocytic syndrome in healthy adults. British Journal o

HIV-Associated Multinucleated Giant Cells in Lymphoid Tissue of the Waldeyer's Ring: A Detailed Study

Huematology, 89, 923-926. (accepted by the Histiocyte Society) have been published (Henter et al, 1991). Unfortunately, in a recent issue Shirono & Tsuda (1995) have confused the hereditary and the reactive forms of HLH. Although the mistake is understandable, it is disturbing because it may cause further diagnostic confusion. The authors erroneously referred to a pair of siblings with the hereditary disease FHL (Henter et a], 1993) as having the reactive condition VAHS. Although these siblings also had titres indicating recent parvovirus infection, it must be made clear that they did not have the secondary syndrome VAHS but, on the contrary, had the familial disease FHL. This diagnostic distinction is very important, because patients with FHL require chemotherapy and bone marrow transplantation (BMT) in order to survive, whereas in VAHS BMT is contraindicated. Both of the children have since died no acceptable BMT donor was found. Thus, again it has to be emphasized that evidence of a (viral) infection in a child with HLH does not exclude that the child may have an underlying hereditary disease, such as the autosomal recessive disease FHL. Instead, a (viral) infection may elicit a bout of FHL in these genetically predisposed individuals (Henter et al, 1993). It is important to bear this in mind when treating a child with HLH, in particular if the disease is progressive, prolonged or recurrent, because the hereditary factor is only rarely known in this recessive disease and a delay in treatment may prove fatal. Departments of Paediatrics, JAN-INGE HENTER Karolinska Hospital, GORAN ELINDER* and *Sachs’ Children’s Hospital, Karolinska Institute, Stockholm, Sweden