Hairy Cell Leukemia: Report of an Unusual Case with Hepatomegaly due to a Large Vascular Tumor of the Liver, Mediastinal Mass and Pleural Effusion Containing Hairy Cells

Abstract Culture studies of peripheral blood mononuclear cells from 7 entirely typical cases of hairy cell leukemia showed that after culture in the presence of PHA for 2--5 days, the predominant cell type changed from E- SIg+ CIg+ gamma FcR+ muFcR+ hairy cells to an E+ SIg- CIg- gamma FcR- muFcR- population of transformed cells derived from hairy cells. Depletion and readdition experiments demonstrated that cell-to-cell contact with T cells was necessary for the phenotypic change, while several observations indicated that the E+ population was not derived from T cells present before culture. The E positivity of the cultured cells was shown to be due to the possession of E receptor not acquired from the culture fluid, but the cells differed from true T cells in lacking both mature and immature T-cell antigens. The relevance of these in vitro observations to the continuing controversy concerning the nature of the hairy cell and to the in vivo fluctuations in immunologic phenotype not infrequently observed in hairy cell leukemia is briefly discussed.

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Cell markers in hairy cell leukemia studied in cells from 51 patients

Blood ◽

10.1182/blood.v59.1.52.52 ◽

1982 ◽

Vol 59 (1) ◽

pp. 52-60 ◽

Cited By ~ 71

Author(s):

J Jansen ◽

HR Schuit ◽

CJ Meijer ◽

JA van Nieuwkoop ◽

W Hijmans

Keyword(s):

Heavy Chain ◽

Hairy Cell Leukemia ◽

Lymphocytic Leukemia ◽

Mature Stage ◽

Hairy Cell ◽

Cell Leukemia ◽

Neoplastic Cells ◽

Hairy Cells ◽

Immunoglobulin Levels ◽

Chain Type

Abstract To determine the maturation arrest of the neoplastic cells of hairy- cell leukemia (HCL) and the spectrum of the surface markers on these cells, a series of 51 patients with this disease was studied. The cells of all but two of the patients showed monoclonal surface Ig with respect to light chains. In about one-third of the cases, only gamma heavy chain determinants were present on the cells; the majority carried multiple heavy chain determinants as documented by the application of different fluorochromes. Two patients each showed two different clones of cells, both of the same light chain type. In one of these two patients, two paraproteins were present in the serum. Intracytoplasmic Ig was found in only 4 of 39 cases, in all instances being IgM. All cases studied concerned cells with FclgG receptors; however, the density of this receptor varied. FcIgM receptors also showed a spectrum of density, with some cases showing very few FcIgM- positive cells. Receptors C3 were not observed on the hairy cells. Serum immunoglobulin levels were normal or increased. Paraproteins were found in the sera of 4 of 38 patients. These data suggest that HCL is a neoplasm of B lymphocytes. The neoplastic cells are probably arrested at a more mature stage than the cells of chronic lymphocytic leukemia. The multiple isotypes on the cells indicate a block at the “switch” phase from the small micro-carrying lymphocyte to the larger Ig- producing lymphocyte or plasma cell.

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Hairy cell leukemia: a tumor of pre-plasma cells

Blood ◽

10.1182/blood.v65.3.620.bloodjournal653620 ◽

1985 ◽

Vol 65 (3) ◽

pp. 620-629 ◽

Cited By ~ 1

Author(s):

KC Anderson ◽

AW Boyd ◽

DC Fisher ◽

D Leslie ◽

SF Schlossman ◽

...

Keyword(s):

T Cell ◽

B Cell ◽

Plasma Cell ◽

Hairy Cell Leukemia ◽

Plasma Cells ◽

Tartrate Resistant Acid Phosphatase ◽

Hairy Cell ◽

Cell Leukemia ◽

Cell Surface Antigens ◽

Hairy Cells

Monoclonal antibodies defining B-, T-, and myeloid-restricted cell surface antigens were used to characterize the lineage and state of differentiation of tumor cells isolated from 22 patients with hairy cell leukemia (HCL). These tumors were shown to be of B lineage because they strongly expressed the B cell-restricted antigens B1 and B4 and lacked T cell- and monocyte-restricted antigens. Moreover, the strong expression of the plasma cell-associated PCA-1 antigen on the majority of hairy cells suggested that these tumors correspond to later stages of B cell ontogeny. Dual fluorescence experiments further confirmed that HCL splenocytes that coexpressed B1 and PCA-1 demonstrated both the morphology and tartrate-resistant acid phosphatase positivity of hairy cells. The observation that some hairy cells either spontaneously produce immunoglobulin (Ig) or could be induced to proliferate and secrete Ig provides complementary support for the view that HCL is a pre-plasma cell tumor. However, staining of hairy cells with anti-IL2R1 monoclonal antibody, which is directed to the T cell growth factor receptor and/or with the anti-Mo1 reagent, directed to C3bi complement receptor, distinguish these cells from currently identified B cells.

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Monoclonal antibodies reactive with hairy cell leukemia [see comments]

Blood ◽

10.1182/blood.v74.1.320.bloodjournal741320 ◽

1989 ◽

Vol 74 (1) ◽

pp. 320-325 ◽

Cited By ~ 1

Author(s):

L Visser ◽

A Shaw ◽

J Slupsky ◽

H Vos ◽

S Poppema

Keyword(s):

Monoclonal Antibodies ◽

B Cell ◽

Hairy Cell Leukemia ◽

Small Population ◽

Lymphocytic Leukemia ◽

Hairy Cell ◽

Cell Leukemia ◽

Hairy Cells ◽

A Minor ◽

Insight Into

Monoclonal antibodies reactive with hairy cell leukemia were developed to aid in the diagnosis of this subtype of B cell chronic lymphocytic leukemia and to gain better insight into the origin of hairy cells. Three antibodies were found to be of value in the diagnosis of hairy cell leukemia. Antibody B-ly 2 can be considered a pan-B cell reagent and generally reacts similar to CD22 antibodies. Antibody B-ly 6 is reactive with the same antigen as CD11c (p150/95), an antigen that is present on hairy cell leukemia, macrophages, and a minor subpopulation of lymphocytes. Antibody B-ly 7 is a unique antibody reactive with 144 Kd antigen present only on hairy cell leukemia and a very small population of normal B lymphocytes. This subpopulation may be the counterpart of hairy cells.

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Low-dose deoxycoformycin in the treatment of hairy cell leukemia

Blood ◽

10.1182/blood.v68.5.1119.1119 ◽

1986 ◽

Vol 68 (5) ◽

pp. 1119-1122 ◽

Cited By ~ 75

Author(s):

EH Kraut ◽

BA Bouroncle ◽

MR Grever

Keyword(s):

Bone Marrow ◽

Complete Remission ◽

Hairy Cell Leukemia ◽

Low Dose ◽

Intravenous Bolus ◽

Hairy Cell ◽

Cell Leukemia ◽

Normal Peripheral Blood ◽

Hairy Cells ◽

Reversible Reduction

Abstract Ten patients with progressive hairy cell leukemia were treated with 2′deoxycoformycin (dCF) by intravenous bolus (4 mg/m2) given every other week. All ten patients are evaluable for response and nine of the ten patients have achieved a complete remission. In addition to clearing of hairy cells from the bone marrow, eight patients had resolution of their monocytopenia. Seven of the nine patients remain in unmaintained remission with a median duration of 6.2 months. Two patients have had relapse in the bone marrow alone and continue to have normal peripheral blood counts. They are being followed without treatment. Toxicity was minimal at this low dose with one patient having a mild reversible reduction in creatinine clearance. Four other patients had reversible neutropenia. There were no significant infections associated with treatment. Low-dose deoxycoformycin administered intravenously every other week represents an extremely effective treatment for hairy cell leukemia.

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Synthesis of a peroxidase activity by the cells of hairy cell leukemia: a study by ultrastructural cytochemistry

Blood ◽

10.1182/blood.v52.3.537.537 ◽

1978 ◽

Vol 52 (3) ◽

pp. 537-550 ◽

Cited By ~ 31

Author(s):

F Reyes ◽

MF Gourdin ◽

JP Farcet ◽

B Dreyfus ◽

J Breton-Gorius

Keyword(s):

Peroxidase Activity ◽

Hairy Cell Leukemia ◽

Cell Types ◽

Human Monocytes ◽

Hairy Cell ◽

Blood Monocytes ◽

Cell Leukemia ◽

Data Points ◽

Normal Human ◽

Hairy Cells

Abstract The nature of cells present in the blood, marrow, and spleen of patients with hairy cell leukemia is largely debated. These cells have been tentatively categorized on the basis of either monocytic or lymphocytic markers, and the accumulating data points to the fact that they share some characteristics of both cell types. Although hairy cells are known to lack myeloperoxidase-positive granules, present in normal human monocytes, we investigated the possible presence of other peroxidase activities differing from the granule-bound myeloperoxidase. The study was carried out with several methods based on the incubation of fixed and unfixed cells in the presence of diaminobenzidine and hydrogen peroxide. A peroxidase activity was found in hairy cells, located always in the endoplasmic reticulum but not in the Golgi apparatus or in any granule. By its cytochemical characteristics it appears to be closely related to that of tissue macrophages, activated blood monocytes, and other nonlymphocytic hematopoietic cells. This peroxidase is not found in lymphocytes with B or T phenotypes.

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Ultrastructural Modifications in one Case of Hairy Cell Leukemia during Alpha-Interferon Therapy

Tumori Journal ◽

10.1177/030089169207800309 ◽

1992 ◽

Vol 78 (3) ◽

pp. 190-197 ◽

Cited By ~ 6

Author(s):

Manrico Morroni ◽

Giordano Ripa ◽

Guido Bolognesi ◽

Pietro Leoni ◽

Saverio Cinti

Keyword(s):

Endoplasmic Reticulum ◽

Rough Endoplasmic Reticulum ◽

Nuclear Membrane ◽

Hairy Cell Leukemia ◽

Interferon Therapy ◽

Close Contact ◽

Hairy Cell ◽

Cell Leukemia ◽

Ultrastructural Modifications ◽

Hairy Cells

There are many reports concerning the morphology of hairy cell leukemia (HCL), but, to our knowledge, there are no data on the ultrastructural modifications of HCL during interferon therapy. The ultrastructural modifications of neoplastic cells In peripheral blood in a case of HCL were investigated before and 2 and 4 months after beginning treatment with human lymphoblastoid alpha-interferon. Before therapy, hairy cells displayed the typical cytoplasmic projections, and 4 % contained ribosome-lamellae complexes (RLC) (the cells contained up to 7 RLC). Two months from the beginning of therapy, hairy cells had shorter projections, RLC had disappeared, and tubuloreticular structures (TRS) had appeared in 2.2 % of the elements. Four months from the beginning of therapy, TRS persisted in 2.3 % of hairy cells, cylindrical confronting cisternae (CCC) appeared in 6.8 % of the cells, and uncommon RLC, in close contact with the rough endoplasmic reticulum and nuclear membrane, were found in 1.5 % of the elements. The cells contained up to 3 RLC. Our data confirm that interferon stimulates the synthesis of TRS and CCC, whereas the reappearance of uncommon forms of RLC could reflect their neosynthesis, possibly related to the interferon therapy. The frequent findings of a close contact between RLC and nuclear membrane support the view that RLC are derived not only from rough endoplasmic reticulum, but also from the nuclear membrane.

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In vivo induction of proteins during therapy of hairy cell leukemia with alpha-interferon

Blood ◽

10.1182/blood.v69.6.1570.1570 ◽

1987 ◽

Vol 69 (6) ◽

pp. 1570-1573 ◽

Cited By ~ 5

Author(s):

BL Samuels ◽

HM Golomb ◽

BH Brownstein

Keyword(s):

Hairy Cell Leukemia ◽

Polyacrylamide Gel Electrophoresis ◽

Specific Protein ◽

Hairy Cell ◽

Cell Leukemia ◽

Biochemical Effect ◽

Ifn Alpha ◽

Hairy Cells

Abstract The mechanism of the antineoplastic effect of interferon (IFN) is not known and may result from direct effects on the neoplastic cells themselves, activation of intermediary effector cells, or a combination of these effects. The synthesis of specific proteins is induced in hairy cells when they are exposed to alpha-IFN in vitro. In particular, the called p80, is markedly induced. We investigated this effect in the hairy cells of seven patients in the leukemic phase of hairy cell leukemia who were being treated with subcutaneous (SC) IFN alpha 2b (r- Hu-IFN-alpha 2). Polyacrylamide gel electrophoresis (PAGE) was carried out on [35S]methionine-labeled whole cell lysates visualized by autoradiography and silver staining. Within 2 days of starting IFN therapy, induction of specific protein synthesis, including p80 was seen by [35S]methionine labeling in freshly isolated circulating hairy cells from 6 of 6 patients tested. Therefore, alpha-IFN has a direct biochemical effect on hairy cells in vivo that is similar to in vitro effects, at least with regard to p80 synthesis, although the kinetics of this effect may vary in the two situations.

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Treatment of hairy cell leukemia with alternating cycles of pentostatin and recombinant leukocyte A interferon: results of a phase II study.

Journal of Clinical Oncology ◽

10.1200/jco.1990.8.4.721 ◽

1990 ◽

Vol 8 (4) ◽

pp. 721-730 ◽

Cited By ~ 22

Author(s):

A Martin ◽

S Nerenstone ◽

W J Urba ◽

D L Longo ◽

J B Lawrence ◽

...

Keyword(s):

Bone Marrow ◽

Hairy Cell Leukemia ◽

Pathologic Complete Response ◽

Hairy Cell ◽

Cell Leukemia ◽

Cell Infiltrate ◽

Recombinant Interferon ◽

Bone Marrow Biopsies ◽

Hairy Cells

Fifteen patients with hairy cell leukemia (HCL) were treated with deoxycoformycin (pentostatin; dCF) (4 mg/m2 intravenous [IV] every week x 3) and recombinant interferon-alpha 2a (rIFN-alpha 2a) (3 x 10(6) units subcutaneously [SC] daily x 4 weeks) in alternating months for a total of 14 months. Eleven patients had undergone splenectomy; four had received prior systemic therapy with chlorambucil and/or steroids. All 15 are evaluable for toxicity and peripheral blood response, while 14 are assessable for bone marrow response. Toxicity was tolerable with grade 3 or 4 nausea and vomiting in three patients, neutropenic fevers in five, transient but significant depression in eight, and localized cutaneous herpes zoster in four. Circulating hairy cells were undetectable by the end of the first month in 10 of 13 patients, and by the end of the second month in the other three. Fourteen patients had bilateral bone marrow biopsies performed at baseline after 6 months of treatment, at the end of treatment (14 months), and at 6-month intervals during follow-up. Before treatment, all patients had hypercellular marrows with hairy cels replacing normal marrow elements; all showed at least a 95% clearing of their hairy cell infiltrate by 6 months of therapy. However, small collections of residual hairy cells could be detected intermittently on at least one side of bilateral samples in all patients. All patients have completed treatment with a median duration of follow-up off therapy of 27 months (range, 15 to 31 months). To date, all peripheral counts and serum soluble interleukin-2 receptor (sIL2R) levels remain stable, and no patient has had progression of the hairy cell infiltrate in the bone marrow. Although no patient achieved a pathologic complete response, alternating monthly cycles of dCF and rIFN-alpha 2a produced durable partial remissions (PRs) in all patients. Continued follow-up is required to determine the length of such remissions.

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