A small cell variant comprises 20% of all T-PLL cases, and the Sézary cell-like (cerebriform) variant is seen in 5% of cases.Marrow involvement is typically diffuse with morphology similar to what is observed in peripheral blood. In the spleen, the leukemic cell infiltrate both the red pulp and white pulp, and lymph node involvement is typically diffuse through the paracortex. Skin infiltrates are seen in 20% of patients, and the infiltrates are usually dense and confined to the dermis and around the skin appendages. Immunophenotype
T-PLL has the immunophenotype of a mature (post-thymic) T-lymphocyte, and the neoplastic cells are typically positive for pan-T antigens CD2, CD3, and CD7 and negative for TdT and CD1a. The immunophenotype CD4+/CD8- is present in 60% of cases, the CD4+/CD8+ immunophenotype is present in 25%, and the CD4-/CD8+ immunophenotype is present in 15% of cases. Genetic findings
Clonal TCR gene rearrangements for the γ and δ chains are typically found. The most frequent chromosomal abnormality is the inversion of chromosome 14, specifically inv 14(q11;q32). This is found in 80% of cases, while 10% of cases show a reciprocal translocation of chromosome 14 (t(14;14)(q11;q32)). Also, abnormalities of chromosome 8 are seen approximately 75% of patients, including idic (8p11), t(8;8)(p11-12;q12), and trisomy 8. Treatment
Most patients with T-cell prolymphocytic leukemia require immediate treatment.T-cell prolymphocytic leukemia is difficult to treat, and it does not respond to most available chemotherapeutic drugs.
Vaccine already held by practitioners could still be administered up to the expiration date (none expired later than November 2020).The U.S. Centers for Disease Control and Prevention (CDC) recommends that healthy adults 50 years and older get two doses of Shingrix, at least two months apart. Initial clinical trials only tested a gap of less than six months between doses, but unexpected popularity and resulting shortages caused further testing to validate wider spacing of the two doses. Shingrix, which provides strong protection against shingles and PHN, was preferred over Zostavax before Zostavax was discontinued.The zoster vaccine is covered by Medicare Part D. In 2019, more than 90% of Medicare Part D vaccine spending was for the zoster vaccine. 5.8 million vaccine doses were administered to Part D beneficiaries that year at a cost of $857 million. References
Further reading
World Health Organization (2014). "Varicella and herpes zoster vaccines : WHO position paper, June 2014" (PDF). Weekly Epidemiological Record. 89 (25): 265–287. hdl:10665/242227. PMID 24983077. Hamborsky J, Kroger A, Wolfe S, eds. (2015). "Chapter 22: Varicella". Epidemiology and Prevention of Vaccine-Preventable Diseases (13th ed.). Washington D.C.: U.S. Centers for Disease Control and Prevention (CDC). ISBN 978-0990449119. External links
Zostavax Product Page U.S. Food and Drug Administration (FDA)
Shingrix Product Page U.S. Food and Drug Administration (FDA)
"Shingrix Vaccine Information Statement". U.S. Centers for Disease Control and Prevention (CDC). 29 July 2021. "Zostavax (Herpes Zoster Vaccine) Questions and Answers". Questions about Vaccines. U.S. Food and Drug Administration (FDA). 18 February 2021.