title: Importance of early splenectomy in patients with hepatosplenic T-cell lymphoma and severe thrombocytopenia.
abstract: OBJECTIVE
  T-cell lymphomas (TCLs) classically have a poorer response to therapy when compared with B-cell lymphomas and account for only 10-15% of all lymphoid malignancies. Hepatosplenic TCLs are a rare subset of this group that usually present with hepatosplenomegaly, B-symptoms, and only rarely with lymphadenopathy.
  
  
  BACKGROUND
  This disease process, also known as gamma/delta (gamma/delta) TCL because of the expression of the T-cell receptor gamma/delta chain, tends to present in young male patients. Hepatosplenic TCLs have recently gained notoriety because of the realization that patients with long-term treatment of some immunomodulators can develop this potentially fatal disease. These facts are exacerbated by the fact that patients with this disease rarely enjoy remissions of more than brief duration with common chemotherapeutic agents or bone marrow transplants. A novel agent, pralatrexate, has recently been found to have a dramatic activity in this patient population with refractory/relapsed disease. Unfortunately, patients with hepatosplenic TCL often present with thrombocytopenia and this new agent is contraindicated in these patients because of the potential exacerbation of thrombocytopenia with this agent.
  
  
  METHODS
  Because of this we attempted a laparoscopic-assisted splenectomy in one patient with grade 4 thrombocytopenia.
  
  
  RESULTS
  Postoperatively the patient's thrombocytopenia resolved, permitting him to begin treatment with this potentially life-saving agent.
  
  
  CONCLUSIONS
  Due to the lethality of this disease and potential efficacy of new therapies, we believe splenectomy should be considered in patients with hepatosplenic lymphoma in an effort to improve the treatment options and survival of patients with this challenging disease.
authors.affiliation: Department of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, PA, USA. aagumbs@gmail.com
authors.forename: Andrew A
authors.initials: AA
authors.lastname: Gumbs
authors.forename: Jasmine
authors.initials: J
authors.lastname: Zain
authors.forename: Ellen
authors.initials: E
authors.lastname: Neylon
authors.forename: Barbara
authors.initials: B
authors.lastname: MacGregor-Cortelli
authors.forename: Molly
authors.initials: M
authors.lastname: Patterson
authors.forename: Owen A
authors.initials: OA
authors.lastname: O'Connor
mesh_terms: D000630:Aminopterin / Q000031:analogs & derivatives / Q000627:therapeutic use; D017809:Fatal Outcome; D006401:Hematologic Agents / Q000627:therapeutic use; D006801:Humans; D010535:Laparoscopy; D008113:Liver Neoplasms / Q000175:diagnosis / Q000601:surgery*; D016399:Lymphoma, T-Cell / Q000175:diagnosis / Q000601:surgery*; D008297:Male; D008875:Middle Aged; D013156:Splenectomy; D013160:Splenic Neoplasms / Q000175:diagnosis / Q000601:surgery*; D013921:Thrombocytopenia / Q000188:drug therapy* / Q000209:etiology; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: C418863:10-propargyl-10-deazaaminopterin; D006401:Hematologic Agents; D000630:Aminopterin