Cryptococcal immune reconstitution inflammatory syndrome following alemtuzumab therapy.

Alemtuzumab is a lymphocyte ablative agent that may cause susceptibility to severe opportunistic infections similar to those seen in AIDS. Pathogen-specific immune reconstitution syndromes can complicate antiretroviral therapy and immune recovery in HIV-infected patients. We present the first reported case of immune reconstitution syndrome associated with T lymphocyte recovery after alemtuzumab therapy.

Periorbital edema as the initial presentation of T-cell prolymphocytic leukemia.

A 57-year-old woman presented with a history of progressive bilateral upper and lower eyelid edema. Laboratory tests revealed T-cell prolymphocytic leukemia. Despite systemic treatment, she died 2 weeks after presentation. This life-threatening disorder should be added to the differential diagnosis of eyelid edema.

Nonmyeloablative peripheral blood stem cell transplant for T-cell prolymphocytic leukaemia complicated by fulminant haemolysis and acute renal failure at engraftment secondary to minor ABO incompatibility.

We present a 54-year-old man who underwent human leucocyte antigen-identical sibling nonmyeloablative peripheral blood stem cell transplant for primary refractory T-cell prolymphocytic leukaemia (T-PLL). His clinical course was complicated by fulminant haemolysis and acute renal failure at the time of engraftment because of minor ABO incompatibility between the donor and the recipient. This case highlights the curative potential of nonmyeloablative transplantation for T-PLL as well as the potential severity of immune haemolysis secondary to minor ABO incompatibility.

Case report: mantle cell lymphoma, prolymphocytoid variant, with leukostasis syndrome.

A 76-year-old man presented with leukostasis syndrome, including oculodynia, blurred vision, and visual field defects, due to mantle cell lymphoma, prolymphocytoid variant, with marked leukocytosis, 1227 x 10(9)/l. He had splenomegaly but no lymphadenopathy or hepatomegaly. The tumor cells were CD5+, CD19+, CD20+, FMC-7+, and kappa light chain restricted. Immunohistochemistry showed expression of p53 and of cyclin D1. Fluorescent in situ hybridization demonstrated t(11;14) with translocation between CYCLIN D1 and the immunoglobulin heavy-chain genes. The patient received leukapheresis and aggressive chemotherapy, but the leukocyte count remained above 100 x 10(9)/l. The patient's condition rapidly deteriorated with lymphomatous infiltration of his lungs and soft tissues, and he expired 6 months after diagnosis. While it is known that mantle cell lymphoma may have a leukemic phase, the degree of leukocytosis in this case exceeds that previously reported in the literature and resulted in a clinical syndrome of leukostasis.

Allogeneic bone marrow transplantation in a patient with T-prolymphocytic leukemia with small-intestinal involvement.

Although T-prolymphocytic leukemia (T-PLL) is characterized by organ infiltration, small-intestinal involvement is rare. We performed an unrelated allogeneic bone marrow transplantation in a patient with T-PLL who had multiple lymphomatous polyposis of the small intestine refractory to combination chemotherapy (cyclophosphamide, vincristine, and prednisolone [COP] and fludarabine plus cyclophosphamide). The patient developed no graft-versus-host disease (GVHD) and remains in complete remission 16 months after the transplantation. T-PLL is usually refractory to chemotherapy and is a T-cell malignancy with poor prognosis. There have been several reports on allogeneic hematopoietic stem-cell transplantation (allo-HSCT) for T-PLL, but none on allo-HSCT for T-PLL patients with intestinal involvement. It is suggested that allo-HSCT may improve the prognosis in patients with T-PLL involving the small intestine.

T-cell prolymphocytic leukemia with hemorrhagic gastrointestinal involvement and a new chromosomal abnormality.

We report a case of T-cell prolymphocytic leukemia in a 56-year-old woman who exhibited hemorrhaging with gastric involvement as the first manifestation. This patient's condition was diagnosed as T-cell prolymphocytic leukemia based on the findings of lymphocytosis, abnormal immunophenotype, hepatosplenomegaly, lymphadenopathy, and cutaneous involvement. Endoscopic examination of the upper gastrointestinal tract revealed hemorrhage from a gastric lesion with histological involvement. Cytogenetic analysis revealed chromosomal abnormalities, 46,XX,der(1), add(1)(p36), that have not previously been described in T-cell prolymphocytic leukemia. In spite of a transient response to chemotherapy, the patient died 15 months after onset of the disease.

[Cutaneous erythematous rash as first manifestation of T-cell prolymphocytic lymphatic leukemia]. / Erupción cutánea eritematosa como primera manifestación de leucemia linfática prolinfocítica de células T.

We present a case of a man 84 years-old, whose presentation feature was a cutaneous inespecific rash, and was diagnosed of T prolymphocytic leukaemia (T-PLL). In this review we analyze actual aspects concerning biology, diagnosis, classification, prognosis and treatment of this rare mature T cell leukaemia.

Concurrent Hodgkin's disease (mixed cellularity type) and T-cell chronic lymphocytic leukemia/prolymphocytic leukemia.

We describe a patient with leukopenic T-cell chronic lymphocytic leukemia/prolymphocytic leukemia (T-CLL/PLL), according to the Revised European-American Classification of Lymphoid Neoplasms. This patient simultaneously developed classic Hodgkin's disease (HD), a combination previously unreported. The leukemic cells were small and mature, did not have cytoplasmic granulation, and appeared similar to B-cell chronic lymphocytic leukemia. Immunophenotyping of the bone marrow-infiltrating cells revealed a postthymic suppressor/cytotoxic phenotype of CD2+, CD3+, CD4, CD5+, CD8+, CD25-, TCR-alpha beta. A lymph node biopsy showed the histological features of HD (mixed cellularity) with infiltrating CD8+ lymphocytes, and immunohistochemical examination revealed the following phenotype of Reed-Sternberg cells: LeuM1/CD15+, BerH2/CD30+, L26/PanB-, UCHL-1/CD45RO-, cyCD3-, CD4, CD8-, CD20-, CD79a-, EMA-, EBER-1+, LMP-1+. Southern blot analysis of the bone marrow and lymph node revealed the same rearrangement of bands of T-cell-receptor genes. Although the HD was treated with chemotherapy that resulted in complete remission, the T-PLL/CLL took an indolent course. This case may suggest the existence of a subtype of T-CLL/PLL with leukopenia and an indolent clinical course. Both diseases were believed to be independent and not a transformation of one to the other.

Cutaneous involvement as the first manifestation in a case of T-cell prolymphocytic leukaemia.

Mature T-cell malignancies of extracutaneous origin are rare disorders. T-cell prolymphocytic leukaemia (T-PLL) is the most common form of all mature T-cell leukaemias in adults. Secondary skill involvement by T-PLL has been reported in 25% of patients. A case of T-PLL which presented with cutaneous infiltration mimicking a cellulitis-like lesion resistant to antibiotic therapy is described. The diagnosis of T-PLL was subsequently fully supported by the clinical, laboratory and cytological findings, as well as by the immunophenotypic study of the skin biopsy. The present case stresses the importance of accurate evaluation of skin lesions in the diagnosis of some haematological conditions and gives additional information about T-PLL such as a previously non-reported cytogenetic abnormality [t(6;6)] and lack of cutaneous lymphocytic-associated antigen expression.

Leukemic pleural effusion in B-cell prolymphocytic leukemia.

The occurrence of leukemic pleural effusion is a rare complication in chronic lymphocytic leukemia and has not been reported in B-cell prolymphocytic leukemia (B-PLL). We report a case of pleural effusion revealing a B-PLL. The diagnosis was made on the cytological and immunological characteristics of cells in the blood and pleural effusion. This patient was treated with fludarabine and was in complete remission after three courses. This observation may have clinical implications for the use of new adenoside nucleotide analogues in symptomatic B-PLL.

Hairy cell leukemia and allied chronic lymphoid leukemias: current knowledge and new therapeutic options.

This review deals mainly with the essentials of hairy cell leukemia (HCL) detailing clinical aspects, laboratory findings and morphology. Rare manifestations of HCL are listed. Newer aspects relating to cytokines, soluble interleukin receptors and TNF are reviewed. Differential diagnosis including HCL-variant, SLVL, PLL and CLL/PLL are discussed. Prognostic factors and in particular therapeutic aspects are detailed with particular emphasis on the new purine analogues Pentostatin and 2-CdA. A list of suggested reading is offered.

Clinical and hematologic response of chronic lymphocytic and prolymphocytic leukemia persisting after allogeneic bone marrow transplantation with the onset of acute graft-versus-host disease: possible role of graft-versus-leukemia.

One patient with refractory B cell chronic lymphocytic leukemia (CLL) and another with refractory B cell prolymphocytic leukemia (PLL) underwent bone marrow transplantation (BMT) from HLA-identical siblings. Circulating malignant cells persisted at high levels in the patient with PLL and there was clinical evidence of disease progression soon after transplant in the patient with CLL. Starting 4-5 weeks post-BMT, cyclosporine was tapered rapidly to stimulate immunologic graft-versus-leukemia (GVL) reactions. There was a fall in the number of malignant cells and reversal of organomegaly with the onset of acute graft-versus-host disease (GVHD). Both patients received conventional doses of corticosteroids for GVHD which also may have contributed to disease response to some extent. Total clearance of the leukemic cells from the peripheral blood was seen in both patients, and clearance of the marrow was seen in the patient with CLL. However, both patients died of complications of severe GVHD. We conclude that GVHD may be associated with a GVL effect after allogeneic BMT for refractory chronic B cell lymphoproliferative diseases. Whether GVL reaction occur in the absence of clinically obvious GVHD after allogeneic BMT for CLL remains to be seen.

Expression of p13MTCP1 is restricted to mature T-cell proliferations with t(X;14) translocations.

T-cell prolymphocytic leukemia (T-PLL), a rare form of mature T-cell leukemias, and ataxia telangiectasia clonal proliferation, a related condition occurring in patients suffering from ataxia telangiectasia, have been associated to translocations involving the 14q32.1 or Xq28 regions, where are located the TCL1 and MTCP1 putative oncogenes, respectively. The MTCP1 gene is involved in the t(X;14)(q28;q11) translocation associated with these T-cell proliferations. Alternative splicing generates type A and B transcripts that potentially encode two entirely distinct proteins; type A transcripts code for a small mitochondrial protein, p8MTCP1, and type B transcripts, containing an additional open reading frame, may code for 107 amino-acid protein, p13MTCP1. The recently cloned TCL1 gene, also involved in translocations and inversions associated with T-cell proliferations, codes for a 14-kD protein that displays significant homology with p13MTCP1. We have generated rabbit antisera against this putative p13MTCP1 protein and screened for expression of p13MTCP1 normal lymphoid tissues and 33 cases of immature and mature lymphoid T-cell proliferations using a sensitive Western blot assay. We also investigated the MTCP1 locus configuration by Southern blot analysis. The p13MTCP1 protein was detected in the three T-cell proliferations with MTCP1 rearrangements because of t(X;14) translocations, but neither in normal resting and activated lymphocytes nor in the other T-cell leukemias. Our data support the hypothesis that p13MTCP1 and p14TCL1 form a new protein family that plays a key role in the pathogenesis of T-PLL and related conditions.

Leukemic meningitis in B-cell prolymphocytic leukemia. A clinical, pathologic, and ultrastructural case study and a review of the literature.

BACKGROUND: Leukemic meningitis is rare in B-chronic lymphocytic leukemia (CLL) and B-prolymphocytic leukemia (PLL); a MEDLINE search for reports published 1960 and after disclosed only nine prior reports. A patient with stable Rai Stage II CLL/PL developed mental status changes. Lumbar puncture revealed a lymphocytic pleocytosis with prolymphocytes containing intracytoplasmic inclusions. METHODS: The patient's cerebrospinal fluid lymphocyte population was analyzed by immunophenotyping and electron microscopy. RESULTS: The studies revealed a clonal population of B prolymphocytes, with typical immunophenotypic and ultrastructural characteristics. The patient was treated with intrathecal chemotherapy with eventual resolution of the cerebrospinal fluid pleocytosis and return to his normal neurologic status. Prior studies also have revealed the efficacy of intrathecal chemotherapy. CONCLUSION: Leukemic meningitis in CLL or PLL is responsive to treatment with intrathecal chemotherapy.