Chapters authored
Interferon-Induced Thrombotic Microangiopathy
Interferon (IFN) is an effective therapy for multiple disorders. An infrequently reported side effect is thrombotic microangiopathy (TMA): thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). We published the first comprehensive review analyzing this association with the following observations: (1) there was a higher incidence of IFN-induced TMA in myeloproliferative disorders (chronic myelogenous leukemia (CML)) than that in nonmalignant disorders (multiple sclerosis (MS), chronic hepatitis C virus infection (HCV)); (2) mean age at diagnosis was 47 years; (3) there was rare association with hairy cell leukemia (HCL), Sezary syndrome (one case each) and no cases reported for polycythemia vera (PV); (4) sex distribution was balanced (exception of higher prevalence in females for MS); (5) TMA was insidious in onset with long incubation periods (average treatment duration 40.4 months); (6) comparative analysis of mean time (months) to onset of TMA ensuing cumulative IFN exposure was: MS 68.6 vs. CML 35.5 vs. HCV 30.4; (7) confirmed TTP (low ADAMTS 13 levels) was associated with the presence of an inhibitor; (8) outcome analysis revealed complete remission in 27 (40%), persistent chronic kidney disease in 28 (42%) and fatality in 12 patients (18%); (9) corticosteroids, plasma exchange (PEX) and rituximab are effective therapies.
Part of the book: Thrombocytopenia
Gamma-Delta T-cell Lymphoma: An Overview
Gamma-delta T-cell lymphomas are very rare and aggressive T-cell neoplasms with complex heterogenicity and diagnostic complexity. Gamma-delta T lymphocytes originate from CD4− CD8− (double negative) thymocytes in the bone marrow and are distinct from alpha beta subtype. Four entities of gamma-delta lymphomas recognized by 2016 WHO classification of lymphoid neoplasms include: hepatosplenic Tγδ lymphoma (HSγδTL), primary cutaneous gamma-delta TCL (PCTCL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) and large granular lymphocytic leukemias (T-LGL). Extensive literature search based on small case series and case reports identifies few more subtypes of gamma-delta T-lymphomas which were not previously classified by World Health Organization. There remains a critical gap in our understanding of the subtypes of gamma-delta T-cell lymphomas and a lack of updated summarization. In this review, we summarize in detail on the classification, biology, heterogenicity, diagnosis, clinical behavior and treatment options of these rare but clinically important entities.
Part of the book: Peripheral T-cell Lymphomas