M2 PHARMA-April 2, 2019-Imbrium Therapeutics Gains US FDA Orphan Drug Designation for Tinostamustine for the Treatment of T-cell
Prolymphocytic Leukemia
Case-patient 1 was a 63-year-old woman from France who had been treated for T-cell
prolymphocytic leukemia diagnosed in December 2014.
The majority are B-cell malignancies such as follicular lymphoma (FL), mantle cell lymphoma (MCL), marginal zone lymphoma (MZL),
prolymphocytic leukemia (PLL) and hairy cell leukemia (HCL), which all together represent less than 15% of all lymphoid appearing leukocytoses, and less than 5% express T-cell lineage marker variants.
The diagnostic investigations include bone marrow biopsy to confirm the presence of the typical hairy cells and to exclude other diagnoses, such as splenic marginal zone lymphoma or B-cell
prolymphocytic leukemia.
Data were collected in April 2014 and variables occupation and histological type were considered for all leukemia types (acute leukemia, leukemic, basophilic, hairy cells, chronic, Burkitt cells, lymphosarcomatous cells, mast cells, plasma cells, eosinophilic, acute lymphoblastic, chronic lymphoblastic, aleukemic lymphoid, sub-acute lymphoid, lymphoid, acute megakaryoblastic, acute myeloid, aleukemic myeloid, chronic myeloid, sub-acute myeloid, myeloid, acute myelomonocytic, chronic myelomonocytic, acute monocytic, chronic monocytic, aleukemic monocytic, sub-acute monocytic, moncytic,
prolymphocytic, acute promyelocytic, sub-acute, leukemia, adult T-cell lymphoma leukemia).
Apart from HCL, expression of CD103 is seen in HCL variant, a subset of splenic marginal zone lymphomas and rare cases morphologically classified as splenic red pulp lymphoma with villous lymphocytes,
prolymphocytic leukemia, and diffuse large B-cell lymphoma.
Butt et al., "T-cell
prolymphocytic leukemia with extensive cardiovascular infiltrate: leading to multiple myocardial infarctions and cardiac death," Texas Heart Institute Journal, vol.
Moreover, 5-10% of CLL patients may have their disease transformed to a more aggressive large cell lymphoma (Richter's transformation or Richter's syndrome, RS) [12,13] or
prolymphocytic leukemia [14], whereas transformation to PBL is extremely rare.
Alemtuzumab is indicated in the therapy of CLL, T-cell
prolymphocytic leukemia, cutaneous T-cell lymphoma, and peripheral T-cell lymphoma [31].
The acute and chronic myeloid leukemias (CMLs), precursor lymphomas, acute lymphoblastic leukemias- (ALL-)B lymphoblastic leukemia/ lymphoma, and T lymphoblastic leukemia/lymphoma originate in hematopoietic stem or progenitor cells while the majority of lymphomas (NHL, Hodgkin lymphoma, and Burkitt lymphoma), all myelomas, as well as several rare leukemias/lymphomas (adult T-cell leukemia,
prolymphocytic leukemia, and hairy cell leukemia), and one common (CLL) leukemia originate in mature lymphoid cells [55, 114].
More than 55% prolymphocytes in the blood suggests a diagnosis of B-cell
prolymphocytic leukemia (B-PLL) (7).