Adrian Duek MD, Emmanuel Lellouche PhD, Sharon Ben Baruch MD, Reut Mashiach BSc, Yafit Segman MD, Gabriel Bryk PhD, Merav Leiba MD
Background: Multiple myeloma (MM) accounts for approximately 10% of hematological malignancies. The monoclonal immunoglobulin G kappa (IgG-κ) daratumumab can bind to CD38 on MM cells and be detected in serum immunofixation (IF), causing pitfalls in M-protein quantification.
Objectives: To determine the efficacy of mitigating the interference of IgG MM treated with daratumumab.
Methods: Levels of Ig, free light chains (FLC) kappa (κ) and lambda (λ), serum protein electrophoresis (SPE)/IF, and Hydrashift 2/4 assays were assessed following manufacturer's instructions in three patients.
Results: Patient 1 was a 70-year-old male diagnosed with IgG-λ MM. The IF distinguished two monoclonal bands (IgG-κ and IgG-λ). With the Hydrashift assay, the daratumumab–anti-daratumumab immune complex shifted the IgG-κ to the α zone, suggesting that the monoclonal IgG-κ band corresponded to daratumumab. Patient 2 was a 63-year-old male with IgG-κ MM who was receiving daratumumab once every other week. SPE/IF assay revealed a faint monoclonal IgG-κ band in the g zone. A stronger monoclonal band was observed after administration. The IgG-κ band disappeared on the Hydrashift assay, while the daratumumab–anti-daratumumab complex appeared as a broad smear in the α-region. Patient 3, a 63-year-old male diagnosed with IgG-λMM, was receiving daratumumab once every other month. The IF assay showed two distinct bands (IgG-κ and IgG-λ) post-daratumumab administration. The shift to the α zone of the IgG-κ bands on the Hydrashift assay confirmed that the additional band observed post-infusion was due to the daratumumab.
Conclusions: The Hydrashift assay can help distinguish daratumumab from endogenous M-spike.
Osnat Zmora MD, Atara Indursky MD, Baruch Klin MD, Sonia Mendlovic MD
Background: Rare incidence cases are part of the routine work of pediatric surgeons. Cecal anomalies in children are an example of such cases.
Objectives: To describe the presentation, workup, management and outcome of rare cecal anomalies in children and to analyze the skills needed for their successful treatment.
Methods: A retrospective chart review was conducted of all cases of cecal anomalies managed by the pediatric surgical service at a tertiary hospital from June 2017 to January 2020. Data regarding demographics, clinical presentation, radiological studies, surgical treatment, pathology, complications, and outcome were collected.
Results: Five cases of cecal anomalies were encountered over a period of 32 months, including a cecal volvulus, cecal duplication, cecal intussusception, and two cecal masses (one ulcerated lipoma and one polyp). All patients, except the patient with cecal duplication, presented acutely and were managed surgically. Long-term follow-up of 17–24 months was unremarkable in all cases.
Conclusions: A wide knowledge base, careful judgment, and creativity enable pediatric surgeons to successfully treat rare conditions such as rare cecal anomalies. These skills should be part of the education of pediatric surgery trainees.
Miri Zektser MD, Anat Rabinovich MD, Uri Grinbaum MD, Tzvi Porges MD, Aya Gozlan MD, Anna Gourevitch MD, Kayed Al-Athamen MD, Orit Barrett MD, Ido Peles MD, Tehila Kaisman-Elbaz MD, Etai Levi MD
Background: Primary central nervous system lymphoma (PCNSL) is a rare aggressive non-Hodgkin's lymphoma. There are limited data on the management of PCNSL outside of clinical trials.
Objectives: To report experience with three main high-dose methotrexate (HDMTX)-based protocols for PCNSL treatment at one medical center.
Methods: We conducted a retrospective review of the medical records of patients diagnosed with PCNSL who were treated at Soroka Medical Center between 2007 and 2019.
Results: The study included 36 patients, median age 64.9 years; 33 patients received a HDMTX backbone induction therapy, 21 (58.3%) received consolidation treatment in addition. In the entire cohort, 25 patients (75.7%) achieved complete remission (CR, CRu-unconfirmed), with mean progression-free survival (PFS) 32 ± 6.9 months and median overall survival (OS) 59.6 ± 12.4 months. More aggressive regiment such as combination of rituximab, HDMTX, cytarabine and thiotepa had better responses 5 (100%) CR, but also a higher incidence of side effects such as neutropenic fever 5 (100%). In subgroup analysis by age (younger vs. older than 60 years), the PFS was 24.2 vs. 9.3 months, and OS was 64.1 vs. 19.4 months, respectively.
Conclusions: A difference in CR and PFS favored a more aggressive protocol, but the toxicity of the multiagent combinations was significantly higher. The prognosis in younger was better than in older patients, with higher rates of CR, PFS, and OS, although not statistically significant. Overall treatment outcomes are encouraging; however, there is a real need for an adaptive approach for older patients and balancing among the effectiveness and side effects.
Ofira Zloto, M.D., Oded Sagiv, M.D., Ayelet Priel, M.D., Tali Cukierman-Yaffe, M.D., Amir Tirosh, M.D. , Nancy Agmon-Levin, M.D., Shiran Madgar, M.D., Tal Serlin, M.D., Halit Winter, M.D. Ruth Huna-Baron, M.D., Tamara Wygnanski-Jaffe, M.D., Guy Ben Simon, M.D.
Background: Little is known about the success of multidisciplinary thyroid eye disease (TED) clinic.
Objectives: To present the characteristics, treatments, and outcomes of patients treated in a multidisciplinary TED clinic.
Methods: A medical record review of all patients who attended a TED clinic was performed. Data included demographics, medical history, laboratory tests, visual function tests, ocular examinations, clinical activity score (CAS), and assessment of quality-of-life (QOL).
Results: Clinic visits included 132 patients seen during 385 appointments at a TED clinic (mean 12 appointments per patient). Management of TED included medical treatments for 48 patients (36.3%) and surgical treatment for 56 (42.4%). There was a positive significant correlation between the CAS and thyroid-stimulating immunoglobulin (TSI) activity at the first visit and at the last follow-up visit (P < 0.01 and P < 0.02, respectively). However, no correlation was found between the CAS and the thyroid-stimulating hormone levels or between the free triiodothyronine (fT3) and fT4 levels at the first or last visit. There was a significant negative correlation between the CAS and color vision (-0.347, P < 0.01, Pearson correlation) at the first visit, but not between the CAS and visual acuity and visual field at either the first or last visit. Changes in the QOL and the CAS scores were significantly negatively correlated (-0.240, P < 0.01).
Conclusions: Treatment and management decisions for TED should be based on multiple parameters including clinical examinations by ophthalmologists and endocrinologists, laboratory tests, and CAS and QOL scores.
Amir Shabtay MD, Ziv Rivak MD, Elena Schleffer MD, Leonid Barski MD