Long-term follow-up of 233 patients with hairy cell leukaemia, treated initially with pentostatin or cladribine, at a median of 16 years from diagnosis.

Hairy cell leukaemia (HCL) was first described 50 years ago. Median survival was then 4 years. The purine analogues, introduced in the 1980s, transformed this prognosis.

Combinations of ZAP-70, CD38 and IGHV mutational status as predictors of time to first treatment in CLL.

ZAP-70, CD38 and IGHV mutations have all been reported to have prognostic impact in chronic lymphocytic leukemia (CLL), both individually and in paired combinations. We aimed to determine whether the combination of all three factors provided more refined prognostic information concerning the treatment-free interval (TFI) from diagnosis. ZAP-70, CD38 and IGHV mutations were evaluated in 142 patients.

The role of rituximab in combination with pentostatin or cladribine for the treatment of recurrent/refractory hairy cell leukemia.

Background: The purine analogs pentostatin and cladribine have revolutionized the treatment of hairy cell leukemia (HCL) with overall responses in greater than 85% of patients and a median progression-free survival of up to 15 years. They continue to be effective at second- and even third-line therapy; however, alternative treatments are needed for patients who are or have become refractory to these agents or whose remissions are shorter with each course of therapy.

Methods: The authors conducted a retrospective review of 8 patients who received pentostatin or cladribine combined concurrently (n = 6 patients) or sequentially (n = 2 patients) with rituximab at second-line therapy (n = 3 patients) and at subsequent lines of therapy (n = 5 patients).

T-cell large granular lymphocyte leukemia: A report on the treatment of 29 patients and a review of the literature.

Background: To the authors' knowledge, there is no standard treatment for patients with T-cell large granular lymphocyte (LGL) leukemia. Available data are limited by patient numbers and coexisting pathologies.

Methods: The authors report on the use of immunosuppressants (cyclosporin A [CSA] and low-dose oral methotrexate [MTX] given continuously) and cytotoxic agents in the treatment of 29 patients with T-cell LGL leukemia age over the past 20 years.

Long remissions in hairy cell leukemia with purine analogs: a report of 219 patients with a median follow-up of 12.5 years.

Background: Both pentostatin and cladribine have efficacy in hairy cell leukemia (HCL), but it is not known which agent achieves better results.

Methods: We reviewed a series of 219 patients with HCL, with median follow-up from diagnosis of 12.5 years (range 1.

The efficacy of alemtuzumab for refractory chronic lymphocytic leukemia in relation to cytogenetic abnormalities of p53.

We reviewed the efficacy of alemtuzumab in the treatment of 28 patients with refractory chronic lymphocytic leukemia (CLL) in whom p53 status was known. Overall responses of 53.6% (complete responses 17.

Zeta-chain associated protein 70 and CD38 combined predict the time to first treatment in patients with chronic lymphocytic leukemia.

Background: Zeta-chain associated protein (ZAP)-70 has been proposed as a surrogate marker for immunoglobulin heavy-chain variable region (IgVH) mutation in chronic lymphocytic leukemia (CLL), but it is still not clear whether it is an independent prognostic factor.

Methods: The authors evaluated ZAP-70 expression by flow cytometry in 201 untreated patients and correlated ZAP-70 levels with CD38 expression, genetic abnormalities detected by fluorescence in situ hybridization (FISH), and the time from diagnosis to first treatment.

Results: Fifty-seven patients (28%) were positive for ZAP-70 (> or = 20%).

Growth factors in haematological cancers.

Since their discovery just under a century ago, growth factors (GFs) have been used almost ubiquitously in haematology. Many haematological cancers are associated with bone marrow failure, either as a direct consequence of the disease or its treatment. Colony stimulating factors (CSFs) have been used to address the problems associated with the resulting cytopenias, however, concerns about the potential leukaemogenic effects of some of these CSFs led to a degree of initial hesitancy in usage, particularly in the management of acute myeloid leukaemia (AML).

Histopathology of the spleen in T-cell large granular lymphocyte leukemia and T-cell prolymphocytic leukemia: a comparative review.

We review retrospectively the spleen histology in 8 patients with T-cell large granular lymphocyte (LGL) leukemia and 4 with T-cell prolymphocytic leukemia (T-PLL) to identify characteristic patterns of involvement and to distinguish such patterns from those described in other low grade B- and T-cell malignancies. Moderate splenic enlargement with red pulp expansion due to lymphocytic infiltration was characteristic of LGL leukemia. Abnormal lymphocytes expressed cytotoxic granule proteins and were consistently CD45RO and CD5 negative in contrast to normal red pulp T cells.

Pregnancy improves neutropenia in T-cell large granular lymphocyte leukaemia.

The effect of pregnancy on T-cell large granular lymphocyte (LGL) leukaemia has not been previously described. We retrospectively reviewed the clinical features of three patients with T-cell LGL leukaemia; each of them had one or more pregnancies during disease evolution. Pregnancy was associated with sustained improvement in neutrophil count and concurrent reduction in lymphocytosis.

Delayed response to fludarabine in lymphoplasmacytic lymphoma/Waldenström's macroglobulinemia.

We retrospectively reviewed time to response and incidence of delayed responses in 13 patients with lymphoplasmacytic lymphoma/Waldenstrom's macroglobulinemia (LPL/WM) treated with fludarabine with or without cyclophosphamide. During follow-up post-treatment, seven delayed responses (54%) were observed, improving the initial overall response rate of 61% to a final response rate of 77%.

Lessons from a case of T-cell large granular lymphocytic leukaemia suggesting that immunomodulatory therapy is more effective than intensive treatment.

Leukemia treatment strives to eradicate the malignant clone. With T-cell large granular lymphocytic (LGL) leukemia, the onus of treatment appears to be modification of the disease rather than eradication of the clone. We describe a case of T-cell LGL leukemia where aggressive, eradicative type therapy proved ineffective.

Granulomatous slack skin disease--disease features and response to pentostatin.

Granulomatous slack skin disease (GSSD) is a rare condition characterized clinically by redundant skin folds, which show a predilection towards flexural areas, and histologically by a granulomatous T-cell infiltrate and loss of elastic fibres. The disease is often indolent, although rapid progression and transformation have been described. There is much debate as to whether this condition is a subset of mycosis fungoides or a separate disease entity in itself.