[A case of atypical hairy cell leukemia--hairy cells expressed Leu 1 antigen after treatment with TPA].
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In vitro studies on leukemia cells and T lymphocytes in hairy cell leukemia.
Hairy cell leukemia cell lines were established from eight untreated patients using purified B cell growth factor (BCGF) in vitro. These cell lines maintained their original cell surface immunophenotype for about 1 month, after which they began to lose one or more of their characteristic surface antigens. The cell lines also maintained typical hairy cell leukemia morphology for 2-3 months in vitro but later showed an increasing number of multinucleate giant cells that maintained a B cell surface phenotype. The cell lines became independent of exogenously provided BCGF after at least 1 month in vitro and secreted BCGF activity into culture supernatants in most cases. Some cell lines also acquired Epstein-Barr virus nuclear antigen positivity after variable period. Two hairy cell leukemia patients also showed hyperactive T cell responses in vitro and exhibited spontaneous T cell proliferation in culture without exogenously supplied interleukin-2. These T cell lines had the T helper phenotype and secreted significant amounts of T cell-associated lymphokines with BCGF and interleukin-2 activity into culture supernatants.
Antibodies, Monoclonal↗
Current treatment options in hairy cell leukemia and hairy cell leukemia variant.
Hairy cell leukemia (HCL) is a chronic B-cell lymphoproliferative disorder characterized by splenomegaly, pancytopenia and circulating lymphocytes displaying prominent cytoplasmic projections. HCL has usually an indolent course and the patients with asymptomatic disease do not require therapy. Treatment of progressive symptomatic HCL includes a variety of pharmacological approaches such as interferon-alpha (IFN-alpha), pentostatin (DCF) and cladribine (2-CdA), which have significantly improved the disease prognosis. 2-CdA and DCF seem to induce a similar high response rate and a long overall survival. They are also active in relapsed patients. More recently high activity of anti-CD20 monoclonal antibody (rituximab) and anti-CD25 (LMB-2) and anti-CD22 (BL-22) immunotoxins have increased the number of therapeutic options for HCL. Splenectomy may be still indicated in patients with massive, symptomatic splenomegaly or results in severe cytopenia. IFN-alpha may have a place in patients with very severe cytopenia, in HCL in pregnancy and in patients who have failed prior therapy with purine nucleoside analogs. HCL variant (HCL-V) is a distinct clinico-pathological entity which seems to be resistant to IFN-alpha and purine nucleoside analogs - DCF and 2-CdA. However, preliminary observations suggest that monoclonal antibodies - rituximab and BL-22 immunotoxin are highly active in this disorder even refractory to 2-CdA. In this review current therapeutic strategies in HCL and HCL-V are presented.
Algorithms↗
2-chlorodeoxyadenosine (cladribine) in the treatment of hairy cell leukemia.
Hairy cell leukemia is an uncommon chronic B cell lymphoproliferative disorder in Taiwan and only sporadic cases have been noted. This disease is often found in middle-aged men with the typical manifestations of pancytopenia and splenomegaly. While tartrate-resistant acid phosphatase (TRAP) stain of the neoplastic cells in peripheral blood smear is positive in 90% cases, bone marrow examination is still necessary for diagnosis. For treatment, splenectomy and interferon have been the standard strategies. Recently, newly-developed purine analogues, 2-chlorodeoxyadenosine (cladribine) and 2'-deoxycorfomycin (pentostatin), were found to achieve long lasting complete remission. Here we reported a 48-year-old men who was found to have splenomegaly and bicytopenia incidentally at routine physical checkup. Although the TRAP staining of peripheral blood showed negative result, hairy cell leukemia was finally diagnosed by bone marrow biopsy. The following splenectomy showed a typical picture of hairy cell leukemia on pathology, but cytopenia recovered only partially after surgery. One course of chemotherapy with cladribine (0.1 mg/kg/day continuous intravenous infusion for 7 days) was given after recovery from major surgery. The white cell and platelet counts normalized after treatment and he carried a stable condition thereafter. Since the disease is rare and purine analogues are newly developed, there has been no report on using 2-clorodeoxy-adenosine against hairy cell leukemia in our country. Here we suggest that newly developed purine analogues such as cladribine are the first choice of treatment in cases of hairy cell leukemia.
Antineoplastic Agents↗
Cutaneous findings in hairy cell leukemia.
Hairy cell leukemia is a distinctive clinicopathologic entity that is histologically characterized by infiltration of the spleen, bone marrow, and blood by uniform mononuclear cells--"hairy cells." We reviewed the prevalent cutaneous findings, specific and nonspecific, in 113 cases of hairy cell leukemia. Only one patient had specific lesions (skin infiltration by leukemic cells). Nonspecific cutaneous findings included recurrent infections, ecchymoses, petechiae, pallor, drug reactions and reactions to transfusions, and nonherpetic ulcerations. Although leukemic infiltration of the skin in hairy cell leukemia is rare, cutaneous histopathologic features may be diagnostic, particularly when cytomorphologic examination is coupled with staining of a fresh specimen with tartrate-resistant acid phosphatase. Cutaneous and extracutaneous infections, often extensive and with opportunistic organisms, were the most common nonspecific findings and demonstrated severely deficient immunologic responsiveness. Associated malignant diseases, cutaneous and extracutaneous, were few in number and are of uncertain significance.
Aged↗
Elucidation of the mechanisms of hairy-cell localization in tissues and the process of the bone marrow fibrosis in hairy-cell leukemia.
Hairy cell leukemia is a chronic B cell leukemia with a number of distinctive features including the unusual tissue distribution of the leukemic cells, hairy cells, and the bone marrow fibrosis. We have been working, for a number of years, on the potential mechanisms behind hairy-cell localization in tissues. In this review, it is summarized how our work has shed very important information regarding these mechanisms and led, eventually, to the full elucidation of the process of the bone marrow fibrosis in hairy cell leukemia.
Bone Marrow↗
Leukopenic chronic T cell leukemia mimicking hairy cell leukemia: association with human retroviruses.
We report two cases of a T cell lymphoproliferative disease not previously described, with cytologic and clinical features similar to those associated with Galton's "prolymphocytic" leukemia (PL). Our patients, like those with Galton's PL, had massive splenomegaly and minimal or absent hepatomegaly and lymphadenopathy. In contrast, however, our patients had leukopenia, as well as low percentages of leukemic cells in the peripheral blood and in the bone marrow. In splenic imprints, the nuclear chromatin pattern of most of the leukemic cells was intermediate between those of mature lymphocytes and those of lymphoblasts, and the nuclei contained single, centrally located, conspicuous nucleoli. In sections of the spleen, the leukemic cells diffusely infiltrated the red pulp in a pattern strikingly similar to that of hairy cell leukemia; however, when the leukemic cells were studied cytochemically, the cytoplasmic acid phosphatase positivity was punctate and tartrate-sensitive. The leukemic cells were sheep erythrocyte rosette-positive and expressed T cell-associated antigens. Initially, both patients responded well to therapeutic splenectomy. One patient received combination chemotherapy after splenectomy and is alive and well 24 months after diagnosis. The other patient was in complete clinical remission for one year after splenectomy and received chemotherapy at relapse. He died, however, 23 months after splenectomy, with disseminated disease. IgG antibody titers against human T lymphotropic virus type I (HTLV-I) were detected in one patient and against HTLV-II in the other. The leukemia in these patients represents a distinct clinicopathologic entity within the spectrum of peripheral T cell lymphoproliferative diseases that includes Galton's PL of T cell derivation, T cell chronic lymphocytic leukemia, T cell hairy cell leukemia, and adult T cell leukemia/lymphoma.
Adult↗
Hairy cell leukemia.
Hairy cell leukemia is a malignancy with a variable course that can be relatively indolent or rapidly fatal. Alterations in the immune system are responsible for much of the morbidity and mortality from hairy cell leukemia. More than 60% of patients die from infection, and infections are both pyogenic and nonpyogenic. Many patients have transfusion requirements, and bleeding complications can also occur. Treatment strategies for hairy cell leukemia have evolved and are being modified as more is learned about the disease. Splenectomy is the initial treatment when patients become symptomatic, and if the disease progresses after splenectomy, chlorambucil offers good control in many patients. Radiation can be used for local palliation, as when complications such as bulky adenopathy or bone lesions occur. Initial studies using interferon in the treatment of hairy cell leukemia look encouraging, but more investigation is necessary before the role of interferon in treatment of hairy cell leukemia is determined.
Androgens↗
Phase II trials of pentostatin (Nipent) in hairy cell leukemia.
Hairy cell leukemia (HCL) is a chronic lymphoproliferative disease of B-cell origin manifested by pancytopenia and splenomegaly. Before 1980 the only effective treatment for HCL was splenectomy, which resolved the cytopenia but did not eliminate the disease from the bone marrow. In addition, the majority of patients progressed after splenectomy and required further treatment. Pentostatin (Nipent; SuperGen, San Ramon, CA) is a purine antimetabolite that was found in phase I studies to induce profound lymphocytopenia Although in vitro studies suggested that T lymphocytes were most sensitive to pentostatin, patients with B-cell chronic lymphatic leukemia and low-grade non-Hodgkin's lymphoma responded to treatment in the initial phase I trials. Due to evidence that the drug was effective in lymphoproliferative disease, patients with HCL were treated with pentostatin. The promising initial results led to phase II studies in both untreated and previously treated patients. These studies demonstrated that pentostatin was highly effective as a single agent, with complete responses seen in 60% to 90% of patients. These responses were durable without maintenance chemotherapy and were seen in patients previously treated with interferon or chemotherapy. Toxicity was usually mild, with nausea and skin rashes predominating. When seen, infections resulting from neutropenia occurred early in treatment. The high response rates and low toxicity suggest that pentostatin should be considered as one of the standard treatments for HCL.
Adenosine Deaminase Inhibitors↗
Serum soluble IL-2 receptor as a reliable and noninvasive marker of disease activity in patients with hairy cell leukemia.
Hairy cell leukemia is a chronic lymphoproliferative disorder of B-cell lineage. Malignant cells express the interleukin-2 receptor (IL-2R) which is released in vitro as well as in vivo. The sera of patients with hairy cell leukemia contain elevated levels of this soluble receptor (sIL-2R). Sera of 24 patients with hairy cell leukemia were tested for sIL-2R. In 9 patients treated with 2-chlorodeoxyadenosine an improved clinical status was associated with decreasing serum sIL-2R. The maximal rate of decrease of sIL-2R level was observed within the second and the third week after the therapy initiation. Patients with disease progression had an increase in serum sIL-2R level. Our results suggest that serial measurement of sIL-2R level can be used as a reliable, noninvasive means to assess the disease activity and its response to therapy.
Adult↗
[Retinopathy as the first symptom of hairy cell leukemia].
Hairy cell leukemia is a seldom non-Hodgkin-lymphoma of the B-cells. The case of a 39 year old male with loss of vision as first sign of hairy cell leukemia is presented. The decrease of vision was caused by intraretinal bleeding into the macula. By means of ophthalmoscopy and angiography we found intraretinal bleedings, soft exudates, microaneurysms and capillary occlusions. The examination of blood cells showed leucocytosis (48.900/microliter), anemia (hemoglobin 8.7 g/l), thrombocytopenia (67.000/microliter) and hairy cells in peripheral blood and in bone marrow. Under treatment with alpha-Interferon visual acuity became normal and the fundus lesions decreased. This case points out that a retinopathy with bleedings and soft exudates can be caused by leukemia which should therefore be excluded by differential blood count.
Adult↗
Listeria monocytogenes cerebritis, bacteremia, and cutaneous lesions complicating hairy cell leukemia.
Hairy cell leukemia is a lymphoreticular malignancy characterized by a chronic course and multiple defects in host defense mechanisms. Infections are the major cause of morbidity and mortality in this malignancy. Opportunistic infections due to pathogens normally controlled by cell-mediated immune mechanisms have been increasingly described but have not included listerial infections. This report describes a case of disseminated Listeria monocytogenes infection including the uncommon manifestations of cerebritis and cutaneous lesions in a patient with hairy cell leukemia.
Anti-Bacterial Agents↗
Hairy cell leukemia.
Hairy cell leukemia is a chronic lymphoproliferative disorder that has been recognized as a separate clinical pathologic entity for the last 25 years. After a decade of discussions about the origin of the neoplastic cell, it has now been well established that hairy cells represent a certain, rather mature stage of B-cell differentiation. Evidence for this has been derived from studies using immunophenotyping with monoclonal and polyclonal antibodies, cytochemistry, and immunoglobulin gene rearrangement. For many years, splenectomy was the only therapy of proven value in hairy cell leukemia. For patients who showed insufficient response to the operation, chemotherapy with low-dose alkylating agents was moderately successful, whereas polychemotherapy often resulted in excessive toxicity. More recently, therapy with alpha-interferon has been shown to be very promising, whereas deoxycoformicin may be an attractive alternative. These new advances in immunology and therapy are reviewed.
Autoimmune Diseases↗
Heterogeneous somatic hypermutation status confounds the cell of origin in hairy cell leukemia.
Hairy cell leukemia (HCL) is thought to arise from a post-germinal center (GC) B-cell, however the exact normal counterpart remains unclear. We performed VH gene analysis of 32 HCL cases, revealing somatically mutated VH genes (<98% homology) in 27 cases and unmutated VH genes in five cases, four of which displayed germline VH genes. Intraclonal heterogeneity was evident in the majority of eight mutated HCLs investigated, although at a lower level compared to GC-derived lymphomas. A novel finding of preferential VH3-30 gene usage was detected (19% of HCLs). Our data confounds the postulated post-GC origin in HCL considering (1) the finding of unmutated HCLs, generally correlating with a pre-GC origin, and (2) the presence of intraclonal variation in mutated HCLs. The latter suggests that the transformed B-cell was frozen when it still had an active mutation process, implying a closer relation to the GC than previously assumed. Furthermore, restricted VH3-30 usage indicates that antigen selection could be a promoting factor in HCL development.
Adult↗
Stereologic analysis of the ultrastructure of isolated human T and non-T lymphoid cells. III. Studies in chronic lymphoid leukemias, hairy cell leukemia and some malignant lymphomas.
Malignant cells from 9 cases of chronic lymphocytic leukemia (CLL), 4 cases of prolymphocytic leukemia (PLL), 4 cases of hairy cell leukemia (HCL), 4 cases of malignant lymphoma of centrocytic type and 3 cases of malignant lymphoma of lymphoblastic type (Kiel classification), all examined for T and B markers, were analysed by electron microscopy. Stereological methods were applied to assess relative and absolute values concerning the whole cell and nuclear and cytoplasmic components of the average cell in each population, and comparisons were made between the morphologically and immunologically defined subsets. The CLL-lymphocyte possessed the smallest cell volume, a high nucleo-cytoplasmic volume ratio, densely packed heterochromatin, a small nucleolar volume and a sparse Golgi complex. The 7 B-derived cases were characterized by a larger volume of rough ER and a more irregular plasma membrane than the two T-derived cases, which typically displayed dense granules in paranuclear aggregations. The prolymphocyte could be distinguished from the CLL-cell by its larger cell volume, lower nucleo-cytoplasmic volume ratio, proportionally lesser heterochromatin, typically condensed at the periphery and around a prominent nucleolus. All 4 cases of PLL were of B-nature and shared the features of B-CLL regarding increased rough ER and low content of granules. The hairy cell exhibited the largest cell volume, the lowest nucleo-cytoplasmic volume ratio, an indented nucleus and a remarkable irregular cellular surface with long "hairy" processes. The cytoplasmic inclusions of ribosome-lamella complexes were recorded exclusively in hairy cells, in half of the patients. The centrocyte was characterized by a cell volume of intermediate size, a high nucleo-cytoplasmic volume ratio and the highest degree of nuclear irregularity recorded. The amount of rough ER was considerably less than in B-CLL and B-PLL. Finally, the blast from the cases of lymphoblastic lymphoma, all of T-nature, displayed a smooth plasma membrane, a high euchromatin-heterochromatin volume ratio and dense granules, characteristically clustered in the vicinity of the Golgi complex. Stereology on the ultrastructure of malignant lymphoid cells provides a more accurate characterization and may be helpful in classification.
Adult↗
Phorbol esters and hairy cell leukemia: effects on cell morphology and surface membrane features and comparison with other B cell leukemias.
Hairy cell leukemia (HCL) mononuclear cells were incubated with the phorbol ester TPA in an attempt to induce further maturation and were compared with B cell chronic lymphocytic leukemia, prolymphocytic leukemia, and non-Hodgkin's lymphoma cells. Morphology, surface features, membrane markers, tartrate-resistant acid phosphatase, and Ig secretion were examined. HCL cells spread and adhered firmly after TPA, producing elongated filopodia. Cells still retained ribosomal lamellar complexes, and increased numbers of dense bodies were seen. TPA enhanced the adherent and phagocytic properties of HCL cells, producing a modest increase in the expression of membrane Ig, GP-70, and Leu-M5 markers, tartrate-resistant acid phosphatase, and Ig secretion. Other neoplastic B cells behaved differently, forming readily detachable clumps without elongated filopodia. Maturation to plasma cells and hairy cell features were readily evident in all cases. These differences in growth patterns were consistent and may be used to distinguish HCL from other B cell neoplasias.
Acid Phosphatase↗
[Treatment of hairy cell leukemia].
Hairy cell leukemia is a rare leukemic variant with a relatively good prognosis. Treatment is indicated when the patient develops symptoms caused by cytopenia. When the spleen is palpable, the primary treatment should be splenectomy. When the spleen is not palpable and/or if the patient relapses after splenectomy, treatment with Alfa-Interferon is indicated. The place of deoxycoformycin in the treatment of hairy cell leukemia has not yet been established.
Adult↗
Serum soluble interleukin-2 receptor is associated with clinical and pathologic disease status in hairy cell leukemia.
Hairy cell leukemia is a chronic lymphoproliferative disorder of B-cell lineage, whose malignant cells express the interleukin-2 (IL-2) receptor. A soluble form of the IL-2 receptor is released by these cells in culture, and the sera of patients with hairy cell leukemia contain elevated levels of this soluble receptor. Four hundred twenty-seven serum samples from 101 patients were analyzed for soluble IL-2 receptor (sIL-2R). The clinical status of patients appeared to be associated with the serum level of sIL-2R. The hairy cell index (a measure of tumor cell burden) was correlated with the square root of the serum sIL-2R level (r = .77). Improved clinical status was associated with decreasing serum sIL-2R levels, whereas disease relapse was associated with increasing levels. Notably, every patient who responded to therapy had a decline in serum sIL-2R level, and every patient with disease progression had an increase in serum sIL-2R level. This phenomenon was observed for several different treatments, including standard-dose interferon, low-dose interferon, and deoxycoformycin. The predictive reliability of this test is currently being prospectively evaluated.
Dose-Response Relationship, Drug↗