Pediatric nodal marginal zone lymphoma (NMZL) is described as a separate variant of NMZL in the most recent WHO classification of tumors of hematologic and lymphoid tissues. It has distinctive morphology and clinical presentation and stands out as an indolent disease with remarkably better overall prognosis compared to classic NMZL. Here we report two adult cases of NMZL with clinical and morphologic features consistent with pediatric NMZL (pNMZL) and review available literature describing the clinical and histologic presentation of pNMZL.. Two men, ages 44 and 18 years, each presented with localized cervical lymphadenopathy that histologically demonstrated florid proliferation of the marginal zone and disruption of reactive germinal centers, progressive transformation of germinal centers (PTGC) -like morphologic features typical for pNMZL and clonal disease with immunophenotype consistent with NMZL. This is the first report of pNMZL in a middle-aged person. Distinct histologic features and characteristic benign clinical course will help to distinguish this rare variant from other NMZL in adults. Clinically, recognition is important to understand the true incidence of this rare form in the adult population and to avoid unnecessary overtreatment of this indolent form.
Fox Chase Cancer Center Partners (FCCCP) is a community hospital/academic partnership consisting of 25 hospitals in the Delaware Valley. Originally created in 1986, FCCCP promotes quality community cancer care through education, quality assurance, and access to clinical trial research. An important aspect of quality assurance is a yearly medical oncology audit that benchmarks quality indicators and guidelines and provides a roadmap for quality improvement initiatives in the community oncology clinical office setting. Each year, the FCCCP team and the Partner Medical Oncologists build disease site- and stage-specific indicators based on National Comprehensive Cancer Network (NCCN) Clinical Practice Guidelines in Oncology. Concordance with multiple indicators is assessed on 20 charts from each community practice. A report for each FCCCP medical oncology practice summarizes documentation, screening recommendations, new drug use, and research trends in a particular disease site. Descriptive statistics reflect indicators met, number of new cases seen per year, number of disease site cases from tumor registry information, and clinical trial accrual total. Education and documentation tools are provided to physicians and oncology office nursing staff. The FCCCP Clinical Operations Team, consisting of medical oncologists and oncology-certified nurses, has conducted quality audits in medical oncology offices for 7 years using NCCN-derived indicators. Successful audits comprising gastric, colorectal, and breast cancer have been the focus of recent evaluations. For the 2005 stage II/III breast cancer evaluation, mean compliance per parameter was 88%, with 15 of 16 practices achieving mean compliance greater than 80%. A large-scale quality assurance audit in a community cancer partner network is feasible. Recent evaluation of localized breast cancer shows high compliance with guidelines and identifies areas for focused education. Partnership between academic and community oncologists produces a quality review process that is broadly applicable and adaptable to changing medical knowledge.
Optimal treatment of cutaneous B-cell lymphoma (CBCL) is yet to be established. We treated five patients, each with either extensive lesions, severe comorbidities or who refused treatment with radiation therapy, with rituximab given as a single agent for four weekly intravenous infusions of 375 mg/m2. Maintenance therapy, if initiated, was given at 375 mg/m2 once every 2 - 3 months. Objective clinical responses occurred in all five patients. Three patients have ongoing complete clinical remissions with a median follow-up of 17, 19 and 39 months post achievement of complete remission. One patient died at age 87 years from a non-related cause after 5.5 years of complete remission. One patient received local radiotherapy to a solitary cutaneous site of large-cell lymphoma that developed after 3 years in remission from the low-grade CBCL; no recurrences of either grade CBCL have yet occurred. Treatment was well tolerated. Rituximab is safe and effective in treating of CBCL, either primary CBCL or low-grade lymphomas with relapses limited to the skin. Rituximab appears to present an attractive alternative when radiation therapy is contraindicated or unwanted. Additional collaborative studies are needed to assess the role of rituximab in various clinicopathologic presentations of CBCL.
3529 Hematologic malignancies in older patients are often characterized by resistant phenotypes which would ideally be treated by allogeneic hematopoietic stem cell transplant (HSCT). However, allogeneic HSCT in this age group is associated with greater regimen-related toxicity than in younger patients limiting its application. In addition, older patients have a smaller matched related donor pool due to the age and comorbidities of their siblings, but often have haploidentical offspring donor options. In order to extend the application of HSCT to older patients (>66), we developed a reduced intensity regimen utilizing haploidentical donors. In this approach, donor T cells and CD 34+ cells are infused at 2 separate times so that the dose and timing of each cell type can be independently controlled. This “break apart” method allows the tolerization of donor T cells by cyclophosphamide (CY) without exposure of the donor HSCs to the drug. Seventeen patients ages 66–77 with AML (9), advanced MDS (2), biphenotypic leukemia (1), CLL (2), NHL (2), and myelofibrosis (1) were treated. All but 2 patients had persistent disease at the time of HSCT. Ten of 17 had a Karnofsky Performance Score (KPS) of >90%, while 7 patients' KPS was 70–80%. The hematopoietic cell transplantation comorbidity index (HCT CI) for the group was between 0 and 5 points. Time from first treatment to HSCT was widely variable. The patients received fludarabine 30 mg/m2/d and cytarabine 2 Gms/m2/d on days -11 through -8. Thiotepa 5 mg/m2/d on days -11 to -9 was substituted for cytarabine in patients with active myeloid malignancies at HSCT. TBI (2 Gy) was given on d-6 immediately followed by 2 ×10e8/kg of the donor's T cells (DLI step). This T cell dose was associated with consistent engraftment and acceptable rates of GVHD in our prior studies. CY 60 mg/kg/d was given on days -3 and -2. A CD34 selected donor product was infused on day 0 (HSC step). T cells were collected from the donors prior to the start of G-CSF for stem cell mobilization. Mycophenolate Mofetil and Tacrolimus were started on d-1. All patients received the targeted T cell dose of 2×10e8 cells/kg, and within 24 hours developed fevers (median peak temperature 103.8f), and in many cases diarrhea and rash. All of the symptoms from this alloreaction resolved after the 2nd dose of CY. The median CD34+/kg dose was 3.34 × 10e6/kg (range 1.4–8.94 × 10e6/kg). The median amount of residual (non-tolerized) T cells in the HSC product was 0.24 × 10e4/kg (range 0.03–4.5 × 10e4/kg). One patient developed hypotension during the alloreaction requiring steroids. There were no other unexpected infusion-related reactions. Five patients developed decreases in ejection fraction (EF), not always associated with clinical heart failure, and 5 patients developed atrial dysrhythmias. Two early deaths occurred from sepsis and decreased EF on days +2 and +11, both in patients with KPS of 70–80% but with HCT-CI scores of <3 at the time of transplant. Fourteen of 17 (82%) patients were discharged to home and one (6%) to a skilled nursing facility. After discharge, one patient experienced a late rejection and died of toxicity from a second HSCT. Two patients died from severe GVHD (liver and gut) on days +161 and +90. The remaining patients had grade 0 to II GVHD controlled either with steroids or steroids plus photopheresis. One patient died from pneumonia on day +177. Four patients died from relapsed disease on days +55, +186, +198, and +510. Seven total patients (41%) are alive and without evidence of disease 1 to 30 months (median 19 months) after HSCT. All but 1 of these patients had a KPS of > 90% at transplant and all surviving patients had HCT CI scores of <3. Three of 3 patients with HCT CI scores >3 have died. The only 2 patients without active disease at HSCT are both alive, 19 and 26 months post HSCT. Except for the most recently treated patient who is not yet evaluable, all of these older patients have resumed their baseline activities of daily living. Haploidentical RIC using this 2 Step protocol is a viable treatment option for older adults. Rates of significant GVHD were acceptable and the majority of patients were able to tolerate the transplant procedures and be discharged to their homes. Based on the outcomes of this small group of patients, this type of therapy should be offered to fit senior adults with a low co-morbidity index especially if they have achieved a complete remission with up front therapy but are at high risk for relapse in the absence of HSCT. Disclosures: No relevant conflicts of interest to declare.
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