Hairy cell leukemia variant (HCLv) is a rare B-cell chronic lymphoproliferative disorder with features of the classic HCL but presenting some particularities, a poor response to conventional therapy of classic HCL and a more aggressive course of disease [ncbi.nlm.nih.gov]

HCL-v makes up 10% of all these cases and presents in older patients with more severe disease and higher lymphocytosis. [karger.com]

[Table 1] shows the fraction of patients presenting with various clinical manifestations. Anemia of a variable degree was present in 66% with median haemoglobin (Hb) of 8.6 g% (range of 5-12 g/dl). [indianjcancer.com]

which was present in 92% of cases. [ijpmonline.org]

• Surgical Procedure

  If the spleen is enlarged, it may be removed in a surgical procedure known as splenectomy. This usually causes a remission of the disease. [encyclopedia.com]

• Cutaneous Manifestation

  However, data regarding the treatment of these cutaneous manifestations are lacking, given the rarity of the concomitance of HCL and vasculitic syndromes. [cancerindex.org]

• Aggressive Behavior

  Hairy cell leukemias with unmutated IGHV genes define the minor subset refractory to single agent cladribine and with more aggressive behavior. Blood 2009; 114 : 4696–4702. [nature.com]

  Hairy cell leukemias with unmutated IGHV genes define the minor subset refractory to single-agent cladribine and with more aggressive behavior. Blood. 2009;114(21):4696–4702. 41. Tiacci E, Schiavoni G, Forconi F, et al. [dovepress.com]

  "Hairy cell leukemias with unmutated IGHV genes define the minor subset refractory to single agent cladribine and with more aggressive behavior". Blood. 114 (21): 4696–4702. doi : 10.1182/blood-2009-03-212449. PMID 19667403. ^ "Archived copy" (PDF). [en.wikipedia.org]

All the cases of HCL variant (n=10) and four cases of HCL were excluded from the present study, wherein diagnostic workup was inadequate. Hence, final analysis was done on 28 cases. Detailed clinical features were missing in three cases. [ijpmonline.org]

Precise diagnosis and detailed workup are essential because the clinical profile of HCL can closely mimic that of other chronic B-cell lymphoproliferative disorders that are treated differently. [dovepress.com]

This is, to our knowledge, the first reported patient with HCL variant for whom treatment with rituximab was successful, and this treatment needs further investigation. [ncbi.nlm.nih.gov]

Treatment options depend on: the treatment you had before time since your last treatment your general health and fitness If your first treatment doesn't put HCL-V into remission, you might have a different immunotherapy or targeted drug. [cancerresearchuk.org]

We describe a patient with HCL-v, whose course was previously complicated by pure red cell aplasia who achieved CR after treatment with rituximab. We also briefly review outcomes of treatments used in HCL-v reported in the current literature. [haematologica.org]

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 [ncbi.nlm.nih.gov]

Prognosis HCL has a good prognosis .In a large series 80% of patients survived at 12 years. HCL -V has a poorer prognosis and in the only largest series reported the median survival is 9 years. [atlasgeneticsoncology.org]

Other factors potentially affecting prognosis It has been reported that unmutated rearrangements in CLL correlate with poor prognosis. 61 To determine whether poor prognosis in HCL is more closely related to unmutated than to VH4-34 + rearrangements, [bloodjournal.org]

Indolent clinical course; median survival: 9 years Morbidity related to splenomegaly, hypersplenism, and cytopenias Histologic transformation of disease in 6% B-symptoms, marked lymphocytosis, or lymphadenopathy may indicate transformation Poor prognosis [basicmedicalkey.com]

Differential diagnosis Other low grade non-Hodgkin's lymphomas: immunophenotypes HCL-V: splenic marginal zone lymphoma, B-prolymphocytic leukaemia Prognosis Prolonged remission may follow splenectomy. [e-immunohistochemistry.info]

[…] obsolete Definitions Mature small B-cell neoplasm that primarily involves peripheral blood, bone marrow, and spleen Resembles classical HCL but has atypical laboratory, morphologic, or immunophenotypic features Provisional entity in 2008 WHO classification ETIOLOGY [basicmedicalkey.com]

Oleske D, Golomb HM, Farber MD, et al: A case-control inquiry into the etiology of hairy cell leukemia. Am J Epidemiol 121:675, 1985. 8. Stewart DJ, Keating MJ: Radiation exposure as a possible etiologic factor in hairy cell leukemia. [medtextfree.wordpress.com]

Etiology and Pathogenesis The etiology of HCL has not been determined. [clinicalgate.com]

Pathophysiology Pathogenesis in hairy cell leukemia The etiology of unusual cytoplasmic projections is still unclear. There are numerous hypotheses proposed as a mechanism for hairy cell leukemia. [cancertherapyadvisor.com]

Am J Hematol 1996 Aug;52(4):316-8 Abstract quote Considering that the prevalence of some hematologic malignancies may have a geographic distribution that could be related with its etiology, a group of 2,387 patients with acute leukemia (1,968 adults and [thedoctorsdoctor.com]

[…] bruising or bleeding Pain or a feeling of fullness below the rib s Shortness of breath Weakness or feeling tired Weight loss for no known reason Diagnostic Exams Flow cytometry Immunophenotyping Peripheral blood smear Progression and Transformation None Epidemiology [seer.cancer.gov]

Epidemiology This disease is rare, with fewer than 1 in 10,000 people being diagnosed with HCL during their lives. Although most patients are white males over the age of 50, it has been diagnosed in at least one teenager. [wiki.clinicalflow.com]

Epidemiology It is a rare disorder accounting for approximately 0.4% of chronic lymphoid malignancies and 10% of all HCL cases. The disease affects the elderly population without sexual predominance. The median age of the patients is 71 years. [hairycellleukemia.org]

Synonyms Epidemiology A rare disease, constituting 2% of lymphoid leukaemias. Patients are middle-aged to elderly. Clinical features Bone marrow and splenic involvement predominate, most patients presenting with splenomegaly and pancytopenia. [e-immunohistochemistry.info]

Hairy cell leukaemia: descriptive epidemiology and a case-control study. Staines A, Cartwright RA. Leukaemia Research Fund Centre for Clinical Epidemiology, University of Leeds. [thedoctorsdoctor.com]

Useful For Suggests clinical disorders or settings where the test may be helpful Classification of lymphomas Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test Hairy cell leukemia [mayomedicallaboratories.com]

Pathophysiology Pathogenesis in hairy cell leukemia The etiology of unusual cytoplasmic projections is still unclear. There are numerous hypotheses proposed as a mechanism for hairy cell leukemia. [cancertherapyadvisor.com]

[…] lymphoproliferative disease; this patient later developed hairy cell leukemia (a B cell disease), but HTLV-2 was not found in the hairy cell clones. [13] There is no evidence that HTLV-II causes any sort of hematological malignancy, including HCL. [14] Pathophysiology [en.wikipedia.org]

No dose-limiting toxicities were observed when beginning cladribine and rituximab on the same day, although most patients required short-term steroids to prevent and treat rituximab infusion reactions. [clincancerres.aacrjournals.org]

Even if symptoms disappear, you might need therapy to prevent a relapse. [icdlist.com]

Prevention Since the cause for the disease is unknown and there are no specific risk factors, there is no known prevention. [encyclopedia.com]

Thus, distinguishing it from other B-cell neoplasms can both reassure the patient and physician and prevent excessive therapy. The basic phenotype of HCL is relatively non-specific. [cytometry.org]

[…] is no known prevention. [medical-dictionary.thefreedictionary.com]