scholarly journals Dissection of the bone marrow microenvironment in hairy cell leukaemia identifies prognostic tumour and immune related biomarkers

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Rachel M. Koldej ◽  
Ashvind Prabahran ◽  
Chin Wee Tan ◽  
Ashley P. Ng ◽  
Melissa J. Davis ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Response Duration ◽  
Tumour Microenvironment ◽  
Hairy Cell Leukaemia ◽  
Response To Treatment ◽  
Cell Leukaemia ◽  
Hairy Cell ◽  
Variable Response ◽  
Bioinformatics Pipeline ◽  
Spatial Profiling

AbstractHairy cell leukaemia (HCL) is a rare CD20+ B cell malignancy characterised by rare “hairy” B cells and extensive bone marrow (BM) infiltration. Frontline treatment with the purine analogue cladribine (CDA) results in a highly variable response duration. We hypothesised that analysis of the BM tumour microenvironment would identify prognostic biomarkers of response to CDA. HCL BM immunology pre and post CDA treatment and healthy controls were analysed using Digital Spatial Profiling to assess the expression of 57 proteins using an immunology panel. A bioinformatics pipeline was developed to accommodate the more complex experimental design of a spatially resolved study. Treatment with CDA was associated with the reduction in expression of HCL tumour markers (CD20, CD11c) and increased expression of myeloid markers (CD14, CD68, CD66b, ARG1). Expression of HLA-DR, STING, CTLA4, VISTA, OX40L were dysregulated pre- and post-CDA. Duration of response to treatment was associated with greater reduction in tumour burden and infiltration by CD8 T cells into the BM post-CDA. This is the first study to provide a high multiplex analysis of HCL BM microenvironment demonstrating significant immune dysregulation and identify biomarkers of response to CDA. With validation in future studies, prospective application of these biomarkers could allow early identification and increased monitoring in patients at increased relapse risk post CDA.

Bone marrow granulomas in hairy cell leukaemia following 2-chlorodeoxyadenosine therapy

1994 ◽  
Vol 24 (3) ◽  
pp. 271-273 ◽  
Author(s):  
V. FRANCO ◽  
A.M. FLORENA ◽  
G. QUINTINI ◽  
M. MUSSO
Keyword(s):  
Bone Marrow ◽  
Hairy Cell Leukaemia ◽  
Cell Leukaemia ◽  
Hairy Cell

scholarly journals Characteristics and Treatment of Hairy Cell Leukaemia

2014 ◽  
Vol 61 (1) ◽  
Author(s):  
R. Greksák
Keyword(s):  
Bone Marrow ◽  
Overall Survival ◽  
Residual Disease ◽  
Lymphoproliferative Disease ◽  
Hairy Cell Leukaemia ◽  
Cell Leukaemia ◽  
Hairy Cell ◽  
Morphological Examination ◽  
Purine Analogues ◽  
Term Survival

AbstractHairy cell leukaemia (HCL) is a rare chronic indolent lymphoproliferative disease of B-lymphocytes. It always infiltrates the spleen, bone marrow or other organs and is also present in the peripheral blood in more than 95% of cases. The disease takes its name from its characteristic wrinkled surface with hair-like projections. The prognosis of this infaust diagnosis has dramatically changed since the 1980s through more precise diagnosis and efective treatment with purine analogues (1984, 2-deoxycoformy-cine (2-dCF); 1990, 2-chlorodeoxyadenosine (2-CdA)). In our group of 38 patients, we confrmed the diagnostic possibilities and their accuracy; in addition to morphological examination, these include fow cytometric detection of typical surface CD antigens and immunohistochemical recognition of hairy cells in the trephine biopsy. The treatment percentage in the number of response rates (RRs) (100%), complete remissions (CRs) (79%) and overall survival of patients is comparable with previously known data. Overall survival rate without symptoms of disease for more than 10 years after treatment is 87% (33/38). The presence and quantity of minimal residual disease (MRD) after frst-line treatment in bone marrow determine subsequent progression or relapse of the disease, sometimes even occurring many years after the treatment. Probably some new pharmacotherapeutical possibilities (monoclonal antibodies, immunotoxins, tyrosine kinase inhibitors or inhibitors of B-Raf enzyme) could demonstrate the ability to eliminate MRD and cure it in relapsed patients or patients refractory to purine analogues. HCL has become an oncological disease with a relatively good prognosis and long-term survival after standard treatment with purine analogues despite the persistence of indolent MRD in the bone marrow at varying degree of residual infiltration intensity.

A morphometric study of bone marrow angiogenesis in hairy cell leukaemia with clinicopathological correlations

2003 ◽  
Vol 122 (6) ◽  
pp. 900-910 ◽  
Author(s):  
Penelope Korkolopoulou ◽  
Despina A. Gribabis ◽  
Nikolaos Kavantzas ◽  
Maria K. Angelopoulou ◽  
Marina P. Siakantaris ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Hairy Cell Leukaemia ◽  
Cell Leukaemia ◽  
Morphometric Study ◽  
Hairy Cell ◽  
Clinicopathological Correlations

scholarly journals Two Atypical Cases of Classical Hairy Cell Leukaemia and Hairy Cell Leukaemia Variant: A Case Study

2020 ◽  
Vol 1 ◽  
Author(s):  
S.A.C.D. Ranatunga ◽  
B.L.T. Balasuriya ◽  
C.C. Kariyawasan
Keyword(s):  
Bone Marrow ◽  
B Cell ◽  
Clinical Findings ◽  
Braf V600e Mutation ◽  
Hairy Cell Leukaemia ◽  
Braf V600e ◽  
Full Blood Count ◽  
Cell Leukaemia ◽  
Hairy Cell ◽  
Cervical Lymph Nodes

Introduction: Classical Hairy Cell Leukaemia (cHCL) and Hairy Cell Leukaemia variant (HCL-v) are both rare and slow-growing mature B cell neoplasms. According to flowcytometry data, they fall into the group classified as CD5- CD10- B cell lymphoproliferative disorders. Methods: Two cases with features atypical to two neoplasms at the time of diagnosis were studied. Results: Case 1 was a 15 year old male with right cervical lymph nodes (1x1 cm) in the posterior triangle, a few ecchymotic patches on the arm and a massive splenomegaly. C-reactive protein (CRP) level was 53 mg/dL. Erythrocyte Sedimentation Rate (ESR) was 98 mm/1 st hour. Full Blood Count (FBC) revealed typical features of pancytopenia with monocytopenia. The liver and renal profiles were normal. Morphology of bone marrow was suggestive of cHCL. Flowcytometry and BRAF V600E mutation was positive confirming the diagnosis of cHCL. Case 2 was a 55 year old male presenting with moderate splenomegaly and absolute lymphocytosis. The FBC revealed leukocytosis which is commonly seen with monocytopenia. Blood pictures revealed many hairy cells with moderately basophilic cytoplasm and visible nucleoli suggesting HCL-v. Flowcytometry findings and negative BRAF V600E mutation confirmed HCL-v. Conclusions: Clinical findings, blood images, morphology of bone marrow, flowcytometric findings and positive BRAF V600E mutation confirmed the diagnosis of cHCL in case 1 (15 year old boy) making it as a very rare case. The morphological findings on blood, the presence of characteristic CD markers on flowcytometry and negativity of BRAF V600E confirmed the case 2 as HCL-v, despite having CD10 positivity and monocytopenia.Keywords: Flowcytometric immunophenotyping, Hairy cell leukaemia, Hairy cell leukaemia variant

Bone marrow trephines. Findings in patients with hairy cell leukaemia before and after treatment

1994 ◽  
Vol 25 (2) ◽  
pp. 129-135 ◽  
Author(s):  
S. PITTALUGA ◽  
G. VERHOEF ◽  
A. MAES ◽  
M.A. BOOGAERTS ◽  
C. DE WOLF-PEETERS
Keyword(s):  
Bone Marrow ◽  
Hairy Cell Leukaemia ◽  
Cell Leukaemia ◽  
Hairy Cell ◽  
Before And After

Bone marrow granulomas in hairy cell leukaemia

1996 ◽  
Vol 29 (3) ◽  
pp. 291-291 ◽  
Author(s):  
H.H. Schmidt ◽  
G. Höfler ◽  
Ch. Beham-Schmid ◽  
H. Sill ◽  
W. Linkesch
Keyword(s):  
Bone Marrow ◽  
Hairy Cell Leukaemia ◽  
Cell Leukaemia ◽  
Hairy Cell
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