Images (2)
Hairy cell leukemiaC91.40
Synonym(s)
DefinitionThis section has been translated automatically.
Low-malignant lymphocytic non-Hodgkin's lymphoma of the B-cell type, which shows atypical B-lymphocytes with hair-like extensions, cytochemical evidence of the tartrate-resistant acid phosphatase reaction and mature B-cell markers (CD11c, CD103, CD25) in the blood smear (see also lymphoma, cutaneous B-cell lymphoma).
EtiopathogenesisThis section has been translated automatically.
The disease is probably caused by a mutation in the BRAF gene (V600E).
ManifestationThis section has been translated automatically.
Median age of onset: 60 years; m:f=4:1
Clinical featuresThis section has been translated automatically.
Slowly progressive disease with swelling of the lymph nodes, enlargement of the spleen or liver, anaemia.
Skin and hairy cell leukaemia: Very rare are specific skin manifestations in the form of solitary or multiple, red to red-brown papules and nodules with a smooth surface (Fino P et al. 2012). Furthermore, (non-specific) skin manifestations have been described that develop in the wake of the persistent and progressive immune insufficiency of this patient group:
- Sweet syndrome (Alkayem M et al. 2014).
- Vasculitic syndromes such as: leukocytoclastic vasculitis, panarteritis nodosa.
- Behcet's type skin and mucosal lesions (Oksuz MF et al 2014).
- Tendency to occur UAW (especially arceimite exanthema) especially after chemotherapy (Ganzel C et al. 2012).
- Panniculitides
- Increased occurrence of "banal" but also serious bacterial and viral infections of the skin (Robak E et al. 2021)
LaboratoryThis section has been translated automatically.
Pancytopenia, monocytopenia, in peripheral blood smear: hair cells!
Immunophenotyping: positivity for CD11c, CD15, CD103, CD123
Bone marrow: positivity for CD20, tartrate-resistant acid phosphatase (TRAP), CD72, Annexin A1
HistologyThis section has been translated automatically.
TherapyThis section has been translated automatically.
Treatment of the underlying disease. If BRAF V600E mutation is detected Therapy with Vemurafenib
Progression/forecastThis section has been translated automatically.
Depending on the stage; complete remission can be achieved. Infections are the most frequent cause of death.
LiteratureThis section has been translated automatically.
- Allsup DJ et al (2002) The diagnosis and treatment of hairy-cell leukaemia. Blood Rev 16: 255-262
- Alkayem M et al (2014) A case report of hairy cell leukemia presenting concomitantly with sweet syndrome. Case Rep Med doi: 10.1155/2014/823286.
- Chubar Y et al (2003) Cutaneous reactions in hairy cell leukaemia treated with 2-chlorodeoxyadenosine and allopurinol. Br J Haematol 122: 768-770
- Fino P et al (2012) Skin metastasis in patient with hairy cell leukemia: case report and review of literature. In Vivo 26:311-314
- Ganzel C et al (2012) High incidence of skin rash in patients with hairy cell leukemia treated with cladribine. Leuk Lymphoma 53:1169-1173
- Gollard R et al (1995) The optimal management of hairy cell leukaemia. Drugs 49: 921-931
- Juliusson G et al (1995) Low-dose cladribine for symptomatic hairy cell leukaemia. Br J Haematol 89: 637-639
- Kreitman RJ et al (2003) Immunobiological treatments of hairy-cell leukaemia. Best Pract Res Clin Haematol 16: 117-133
- Mey U et al (2003) Advances in the treatment of hairy-cell leukaemia. Lancet Oncol 4: 86-94
- Oksuz MF et al (2014) Hairy cell leukemia presenting initially with symptoms of Behçet's disease. Int J Rheum Dis 17:689-692
- Robak E et al (2021) Skin changes in hairy cell leukemia. Ann Hematol 100: 615-625.