Hairy cell leukemia with isolated bone lesions

Cailly, Laura; Gruchet, Cécile; Maître, Elsa; Guidez, Stéphanie; Delwail, Vincent; Systchenko, Thomas; Moya, Niels; Sabirou, Florence; Levy, Ayelet; Bobin, Arthur; Gardeney, Helene; Nsiala, Laly; Vonfeld, Mathilde; Chacon, Aurélia; Pichon, Aurélien; Bouyer, Sabrina; Basle, Caroline; Dindinnaud, Elodie; Chomel, Jean-Claude; Rimbault, Anne; Borde, Florence; Troussard, Xavier; Tomowiak, Cécile

doi:10.22541/au.167274624.40434025/v1

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“…During the period of health crisis, the mortality rate related to severe acute respiratory syndrome (SARS)‐coronavirus‐2 (CoV‐2) was rather low even among HCL patients with significant immunosuppression and recent treatment 23 . Autoimmune or unusual clinical manifestations are occasionally reported at diagnosis and/or relapse as bulky abdominal lymph nodes, pleural effusions and ascites, skin lesions 24 or destructive bone lesions, some of which can mimic multiple myeloma 25–27 . With the high frequency of routine PB analyses, hairy cells can also be detected in asymptomatic patients.…”

Section: How the Diagnosis Of Hcl Has Improved In Daily Practice?mentioning

confidence: 99%

“…23 Autoimmune or unusual clinical manifestations are occasionally reported at diagnosis and/or relapse as bulky abdominal lymph nodes, pleural effusions and ascites, skin lesions 24 or destructive bone lesions, some of which can mimic multiple myeloma. [25][26][27] With the high frequency of routine PB analyses, hairy cells can also be detected in asymptomatic patients.…”

Section: How the Diagnosis Of Hcl Has Improved In Daily Practice?mentioning

confidence: 99%

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Hairy cell leukemia 2024: Update on diagnosis, risk‐stratification, and treatment—Annual updates in hematological malignancies

Troussard,

Maître,

Paillassa

2024

American J Hematol

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Disease OverviewHairy cell leukemia (HCL) and HCL‐like disorders, including HCL variant (HCL‐V) and splenic diffuse red pulp lymphoma (SDRPL), are a very heterogenous group of mature lymphoid B‐cell disorders characterized by the identification of hairy cells, a specific genetic profile, a different clinical course and the need for appropriate treatment.DiagnosisDiagnosis of HCL is based on morphological evidence of hairy cells, an HCL immunologic score of 3 or 4 based on the CD11c, CD103, CD123, and CD25 expression, the trephine biopsy which makes it possible to specify the degree of tumoral bone marrow infiltration and the presence of BRAFV600E somatic mutation.Risk StratificationProgression 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. VH4‐34 positive HCL cases are associated with a poor prognosis, as well as HCL with TP53 mutations and HCL‐V.TreatmentPatients should be treated only if HCL is symptomatic. Chemotherapy with risk‐adapted therapy purine analogs (PNAs) are indicated in first‐line HCL patients. The use of chemo‐immunotherapy combining cladribine (CDA) and rituximab (R) represents an increasingly used therapeutic approach. Management of relapsed/refractory disease is based on the use of BRAF inhibitors (BRAFi) plus R, MEK inhibitors (MEKi), recombinant immunoconjugates targeting CD22, Bruton tyrosine kinase inhibitors (BTKi), and Bcl‐2 inhibitors (Bcl‐2i). However, the optimal sequence of the different treatments remains to be determined.

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Section: How the Diagnosis Of Hcl Has Improved In Daily Practice?mentioning

confidence: 99%