Hairy cell leukaemia mimicking multiple myeloma

Notarfranchi, Laura; Russo, Filomena; Mancini, Cristina; Martella, Eugenia; Falini, Brunangelo; Aversa, Franco; Tiacci, Enrico

doi:10.1016/s1470-2045(18)30609-0

Cited by 12 publications

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“…At diagnosis, HCL is usually characterized by infections, splenomegaly or the presence of cytopenias. Autoimmune manifestations or unusual manifestations can be identified, some of which can mimic multiple myeloma …”

Section: How Has the Diagnosis Of Hairy Cell Leukemia And Hcl‐like DImentioning

confidence: 99%

Hairy cell leukemia: 2020 update on diagnosis, risk stratification, and treatment

2019

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Disease overview: Hairy cell leukemia (HCL) and HCL-like disorders, including HCL variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogeneous group of mature lymphoid B-cell disorders. They are characterized by the identification of hairy cells, a specific genetic profile, a different clinical course and the need for appropriate treatment. Diagnosis: Diagnosis of HCL is based on morphological evidence of hairy cells, an HCL immunologic score of three or four based on the CD11C, CD103, CD123, and CD25 expression. Also, the trephine biopsy which makes it possible to specify the degree of tumoral medullary infiltration and the presence of BRAF V600E somatic mutation. Risk stratification: Progression of patients with HCL is based on a large splenomegaly, leukocytosis, a high number of hairy cells in the peripheral blood and the immunoglobulin heavy chain variable region gene mutational status. The VH4-34 positive HCL cases are associated with poor prognosis. Treatment: Risk adapted therapy with purine nucleoside analogs (PNA) are indicated in symptomatic first line HCL patients. The use of PNA followed by rituximab represents an alternative option. Management of progressive or refractory disease is based on the use of BRAF inhibitors associated or not with MEK inhibitors, recombinant immunoconjugates targeting CD22 or BCR inhibitors.

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Section: How Has the Diagnosis Of Hairy Cell Leukemia And Hcl‐like DImentioning

confidence: 99%

Hairy cell leukemia: 2020 update on diagnosis, risk stratification, and treatment

2019

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“…In the largest series reported of eight patients, the median time from initial diagnosis of HCL to the diagnosis of skeletal complications was 20 months (range, 0‐93) 10 . Another study of 36 HCL patients with skeletal lesions found the median time from HCL diagnosis to diagnosis of bone involvement to be 12 months (range 0‐264 months 10‐38 ; in 12 patients (33%), bone lesions were diagnosed at presentation (Table 1). The longest time between HCL diagnosis and bone involvement was 22 years 20 .…”

Section: Resultsmentioning

confidence: 99%

“…Bone involvement is observed in about 3% (0‐13%) of HCL patients 14‐19 . Weh et al describe five cases of bone localization among 150 HCL patients (3.3%), four with femoral neck localization 9 .…”

Section: Resultsmentioning

confidence: 99%

“…Bone involvement is observed in about 3% (0-13%) of HCL patients. [14][15][16][17][18][19] Weh et al describe five cases of bone localization among 150 HCL patients (3.3%), four with femoral neck localization. 9 Lembersky et al found eight cases (3%) of skeletal involvement among 267 HCL patients.…”

Section: Epidemiologymentioning

confidence: 99%

“…However, some reports suggest that they are related to the presence of tumor necrosis factor-alpha (TNF-α), which induces bone resorption. 17,19 TNF-α participates in the autocrine growth control of HCL and has strong bone resorptive properties. 17 In one study, bone mineral density (BMD) was not decreased in 14 patients who did not have bone involvement in radiographic evaluation.…”

Section: Pathogenesismentioning

confidence: 99%

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Bone lesions in hairy cell leukemia: Diagnosis and treatment

Robak

Jesionek-Kupnicka

Kupnicki

et al. 2020

European J of Haematology

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Hairy cell leukemia (HCL) is a rare and indolent form of small mature B-cell leukemias. The annual incidence of HCL is estimated to be 0.3 cases per 100 000, and the disease comprises 2%-3% of all leukemias. 1,2 It was estimated that 1100 new cases of HCL and 4060 deaths occurred in 2016 in the USA. 3,4 It is mostly a disease of middle-aged males (median age 55 years and 4:1 male predominance). Patients present most frequently with splenomegaly, pancytopenia, and bone marrow infiltration. Hairy cells characteristically stain strongly positive for tartrate-resistant acid phosphatase (TRAP) and have a typical pattern of B-cell antigen expression (CD19, CD20) with co-expression of CD11c, CD25, and CD103. Cell surface markers associated with normal B-cell activation, especially CD22, CD72, and CD40, are also strongly expressed. 3,4 The BRAF V600E mutation is present in all patients and is regarded as a disease-defining genetic event. 5 Purine nucleoside analogs (PNA), cladribine (2-CDA), and pentostatin (DCF, deoxycoformycin) are the drugs of choice in first-line treatment of HCL 3,4 ; they induce durable and unmaintained complete response (CR) in more than 70% of patients, with relapse rates of 30%-40%, after 5-to 10-year follow-up. However, despite very high initial response rates, many patients ultimately relapse or develop refractory disease and will require novel therapies after these agents. 3 Unusual clinical manifestations of HCL are occasionally reported. 6-14 These rare symptoms may include bulky abdominal lymphadenopathy, tumor masses in the mediastinum, paravertebral masses,

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Lymphoid Leukaemias of Mature B, T and Natural Killer Cells

2024

Leukaemia Diagnosis

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Hairy cell leukaemia mimicking multiple myeloma

Cited by 12 publications

References 0 publications

Hairy cell leukemia: 2020 update on diagnosis, risk stratification, and treatment

Hairy cell leukemia: 2020 update on diagnosis, risk stratification, and treatment

Bone lesions in hairy cell leukemia: Diagnosis and treatment

Lymphoid Leukaemias of Mature B, T and Natural Killer Cells

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