Anti-PF4 ELISA-Negative, SRA-Positive Heparin-Induced Thrombocytopenia.

Heparin products are frequently used in the inpatient setting to prevent and treat venous thromboembolism, but they simultaneously put patients at risk of developing heparin-induced thrombocytopenia (HIT). The 4Ts score determines the pretest probability of HIT. Diagnosis is made with a screening antiplatelet factor (PF4) immunoassay and the serotonin-release assay (SRA) as a confirmatory test. Anti-PF4 assays have high sensitivity (98%) but lower specificity (50%) and result in frequent false-positive tests. We present a rare case from our institution of a patient with anti-PF4-Polyanion ELISA-negative, SRA-positive HIT and describe the challenges in making a timely diagnosis in this case.

Successful Treatment of Refractory Post-Transplant Lymphoproliferative Disorder With Chimeric Antigen Receptor T-Cell Therapy in a Heart Transplant Recipient.

Post-transplant lymphoproliferative disorders (PTLDs) are opportunistic malignancies that complicate the success of hematopoietic stem cell or solid organ transplantation. These disorders often arise post-transplant due to the immunosuppression required for minimizing the risk of rejection of donor tissue. First-line treatment of these disorders includes limiting immunosuppression when permissible. Subsequent treatment includes the use of monoclonal anti-CD20 antibody (rituximab), and/or combination chemotherapy. Chimeric antigen receptor (CAR) T-cell therapy has revolutionized the treatment paradigm in many lymphoid malignancies. It is not approved for PTLD due to exclusion of PTLD patients from pivotal clinical trials. Also, its utilization post-transplant can be complex and multidisciplinary care is of utmost importance for successful administration of a potentially curative treatment. We present a 68-year-old patient with history of heart transplant for non-ischemic cardiomyopathy, diagnosed with PTLD that was refractory to treatment using current guidelines until successfully receiving CAR T-cell therapy.

The Impact of Renal Impairment in Multiple Myeloma Patients Undergoing Autologous Stem Cell Transplantation With Melphalan Conditioning.

Background: There are no standard renal dose adjustments for melphalan conditioning for autologous stem cell transplantation (ASCT) in multiple myeloma (MM) patients. The objective of this study was to evaluate the effect of melphalan dosing and chronic kidney disease (CKD) on transplant-related outcomes, progression-free survival (PFS), and overall survival (OS). Methods: A retrospective chart review was performed, and MM patients who underwent ASCT between February 2016 and September 2021 were included. Melphalan 200 mg/m2 (Mel200) or 140 mg/m2 (Mel140) was administered. The cohort was divided based on renal function: creatinine clearance (CrCl) ≥ 60 mL/min (no-CKD) and CrCl < 60 mL/min (CKD). Outcomes measured include PFS, OS, treatment-related mortality (TRM), incidence of adverse events, hospitalization duration, and hospital readmission within 30 days. Statistical analysis included Chi-square test, t-test, and Kaplan-Meier method. Logistic regression model was used to account for melphalan dose adjustment. Results: A total of 124 patients were included (n = 108 no-CKD, and n = 16 CKD). Median age was 62 years, majority (62%) were male, and 97% had at least a partial response at time of ASCT. Of the 124 patients, nine (7%) received Mel140. Five of these patients had CKD (CrCl range: 26 - 58 mL/min), with one on hemodialysis. Median time to neutrophil engraftment was 13.6 vs. 14.9 days and median time to platelet engraftment was 18.3 vs. 18.5 days in the CKD group vs. no-CKD group, respectively (P = 0.03 and P = 0.8). When adjusting for melphalan dose reduction, the median time to neutrophil engraftment was not statistically significant (P = 0.11). At a median follow-up of 28.7 months, the median PFS for the CKD vs. no-CKD group was 60 vs. 46 months (P = 0.3). One-year OS was 93.8% in the CKD group vs. 97% in the no-CKD group. There was a higher incidence of grade 3 or 4 mucositis in the CKD group vs. no-CKD group (P = 0.013). Conclusions: There is no significant difference in engraftment, PFS, or OS for MM patients with CKD vs. no-CKD receiving melphalan conditioning for ASCT. Severe mucositis was significantly more common in the CKD group, including when accounting for melphalan dose reduction.

Methemoglobinemia in the Setting of G6PD Deficiency and SARS-CoV-2 Infection.

catalyzes the pentose phosphate shunt. It is required to maintain the level of nicotinamide adenine dinucleotide We report a case of a 58 year old African American male patient with Coronavirus Disease-2019 (COVID-19) in the setting of multiple concomitant hematologic disorders, including Glucose-6-phosphate dehydrogenase deficiency (G6PD deficiency) and sickle cell trait. Typically, G6PD deficiency remains clinically silent, and only a minority of patients will show signs of chronic hemolytic anemia. However, all G6PD deficient patients are at risk of non-immune hemolysis after exposure to a variety of infectious pathogens, including COVID-19. Our patient displayed evidence of methemoglobinemia and subsequent tissue anoxia. We review the theories and mechanisms behind the increased risk of complications and severity of illness in the context of COVID-19 and hematologic disorders. These patients may require alternative treatment pathways due to their comorbidities. This case emphasizes the complications that can arise in this setting, and highlights important considerations for patient treatment.

Donor Cell Leukemia Following Allogeneic Hematopoietic Stem Cell Transplantation.

Approximately 25,000 allogeneic transplants are performed annually worldwide; a figure that has steadily increased over the past three decades. The study of transplant recipient survivorship has become a cogent topic and post-transplant donor cell pathology warrants further study. Donor cell leukemia (DCL) is a rare but serious complication of allogeneic stem cell transplantation (SCT) where the recipient develops a form leukemia originating from the donor cells used for transplantation. Detection of abnormalities predicting donor cell pathology might inform donor selection, and the design of survivorship programs for early detection of these abnormalities might allow therapeutic intervention earlier in the disease course. We present four recipients of allogeneic hematopoietic stem cell transplant (HSCT) from our institution who developed donor cell abnormalities allogeneic SCT, highlighting their clinical characteristics and challenges.

A Meningioma Mimic and Distinct Subtype of Primary Central Nervous System Lymphoma: Primary Dural Lymphoma.

Primary central nervous system lymphoma (PCNSL) is an aggressive form of extranodal non-Hodgkin lymphoma that arises in the brain parenchyma, eyes, meninges, or spinal cord in the absence of systemic disease. Primary dural lymphoma (PDL), in contrast, arises from the dura mater of the brain. PDL is usually a low-grade B-cell marginal zone lymphoma (MZL), whereas other types of PCNSL are usually high-grade large B-cell lymphoma. This specific pathological subtype has important therapeutic and prognostic implications, making PDL a distinct subtype of PCNSL. Herein, we report a case of PDL in an African American patient, in her late thirties, who presented to our emergency room with chronic headaches. An emergent magnetic resonance imaging (MRI) of the brain showed a dural-based homogeneously enhancing extra-axial mass along the left hemisphere, which was contained within the anterior and parietal dural mater. A surgical specimen was collected after an emergency debulking procedure. The flow cytometry, done on the surgical specimen obtained, was positive for CD19+, CD20+, and CD22+, but negative for CD5- and CD10-. These findings were consistent with a clonal B-lymphoproliferative disorder. The surgical pathology specimen immunohistochemistry was positive for CD20+ and CD45+, but negative for Bcl-6Cyclin D1- and CD56-. The Ki67 was 10-20%. These findings were consistent with extranodal MZL. Given the location and pathology, the patient was diagnosed with PDL. Due to MZL's indolent nature, location outside the blood-brain barrier, and known efficacy to bendamustine-rituximab (BR), we decided to treat our patient with BR. She completed six cycles without major complications, and her post-therapy brain MRI showed complete remission (CR). Our case adds to the sparse literature about PDL and highlights the efficacy of BR systemic chemotherapy on MZLs.

Treatment Selection and Survival in Patients with Gray Zone Lymphoma: A Comprehensive Population-Based Analysis.

BACKGROUND AND OBJECTIVES: There are no treatment guidelines for gray-zone lymphoma (GZL), given the disease's rarity and being a relatively new entity. Our objective was to assess factors affecting treatment selection in GZL and its effect on survival, focusing on combined modality treatment (CMT) versus chemotherapy alone. PATIENTS AND METHODS: We identified 1047 patients with GZL treated with CMT or chemotherapy alone between 2004 and 2016 from the National Cancer Database (NCDB). We excluded patients without histologic confirmation of the diagnosis, those who did not receive chemotherapy, and those who started chemotherapy >120 days or radiation >365 days from diagnosis to account for immortal time bias. Factors affecting treatment selection were investigated using a logistic regression model. A propensity score-matched methodology was used to compare survival outcomes. RESULTS: Only 164 patients (15.7%) received CMT, while 883 (84.3%) received chemotherapy alone. Treatment selection was affected by clinical factors (age, odds ratio [OR] 0.99, 95% confidence interval [CI] 0.98-0.997, p-value 0.01 and advanced stage, OR for stage 4: 0.21, 95% CI 0.13-0.34, p-value < 0.001) but not socioeconomic factors. Higher median income was associated with better survival, while increased age, higher comorbidity score, and B symptoms were associated with worse survival. The use of CMT had a survival advantage over chemotherapy alone (hazard ratio [HR] 0.54, 95% CI 0.351-0.833, p-value 0.005). CONCLUSION: CMT is associated with survival advantage in our analysis. Careful selection of patients is essential to achieve the best outcomes with minimal toxicity. Socioeconomic factors affect treatment selection in patients with GZL that can alter outcomes. Future work should focus on strategies that access disparities without compromising survival.

Primary Testicular Lymphoma: Single Center Experience.

BACKGROUND/AIM: Primary testicular lymphoma (PTL) is an exceedingly rare and aggressive form of non-Hodgkin's lymphoma; the most common subtype is diffuse large B-cell (DLBCL). Standard treatment includes orchiectomy, chemotherapy, central nervous system (CNS) prophylaxis, and prophylactic radiation to the contralateral testis. PTL can reoccur years after complete remission. Treatment to immune sanctuary sites, CNS and contralateral testis, is crucial in preventing relapse. There are limited data characterizing this entity and this study aimed to add to existing literature. PATIENTS AND METHODS: This descriptive retrospective study characterized twelve patients with PTL from years 2010-2021 at Allegheny Health Network. Their demographic data, prognostic factors, treatment regimens, and relapse sites (if any) were tabulated. The mean progression-free survival (PFS) was calculated to describe our experience in treating PTL. RESULTS: Twelve patients were diagnosed with PTL; 10/12 (83.33%) patients were diagnosed with ABC PTL-DLBCL. Median age of diagnosis was 67 years. Eight of the 12 (66.66%) were African American, 4/12 (33.33%) were Caucasian. At the time of diagnosis, 8/12 (66.66%) patients presented with an elevated lactate dehydrogenase (LDH) and 8/12 (66.66%) presented with a left testicular mass. Most were treated with R-CHOP (9/12), intrathecal methotrexate (IT-MTX) (10/12), and radiation to the contralateral testis (9/12). Three of the twelve (25%) patients relapsed. Median time to relapse was 8 months. Mean PFS was 50.417 months. CONCLUSION: We discuss our experience in treating PTL with RCHOP, IT-MTX, and irradiation to the contralateral testis and add to the limited pre-existing data that exist.

Venetoclax-induced vitiligo in a patient with chronic lymphocytic leukemia.

Venetoclax, a highly selective Bcl-2 inhibitor, is an orally bioavailable drug that has been approved as first-line therapy for chronic lymphocytic leukemia (CLL) in combination with obinutuzumab, as well as monotherapy in the setting of relapsed CLL. Although some of its life-threatening side effects are well known, including tumor lysis syndrome and cytopenias, others less known side effects include skin reactions. Skin rash is commonly reported in literature, which is often mild and not life-threatening. In this case report, the authors describe what is potentially the second case of venetoclax-induced vitiligo reported in literature. A 77-year-old man with CLL Rai stage II with cytogenetics showed 11 q23 deletion in 14% of cells, and 14q32 partial deletion in 9% of cells developed vitiligo in his extremities 2 years into treatment. A decision was made to continue venetoclax with close monitoring as the side effect was mild and not debilitating. The patient continued to do well. Although vitiligo is not associated with increased mortality risk, its development is associated with increased psychological stress. The mechanism by which vitiligo develops remains unclear. There may be an association between drug-induced vitiligo and improved cancer prognosis; however, larger studies need to be carried out to prove this hypothesis.

Testicular Plasmacytoma Masking as Epididymo-orchitis in a Known Multiple Myeloma Patient.

BACKGROUND/AIM: Extramedullary plasmacytoma (EMP) is defined as a localized plasma cell neoplasm that arises in tissues other than the bone. The most common sites of involvement of EMP are the upper airways followed by lymph nodes, gastrointestinal tract, thyroid gland, skin, brain, liver, and lungs. Testicular plasmacytoma has a very rare occurrence with about 70 cases reported in literature to date. CASE REPORT: We describe a 52-year-old-male with a diagnosis of multiple myeloma presenting with lytic lesions of the axial skeleton. He had lambda light chain restricted, R-ISS stage II with high risk cytogenetics as he tested positive for t(4;14). He underwent four cycles of cyclophosphamide, bortezomib and dexamethasone followed by auto-peripheral stem cell transplantation. He was kept on ixazomib, lenalidomide and dexamethasone maintenance therapy, but relapsed soon after and was diagnosed with plasmacytoma of the left lung. Therapy was switched to daratumumab, carfilzomib and dexamethasone and the patient received radiation of his left lung. He then developed left painless testicular mass which was treated with six weeks course of antibiotics. However due to persistence of concerning features on scrotal ultrasound post-treatment, the patient underwent radical orchiectomy with pathology coming back positive for plasma cells. CONCLUSION: The testes serve as a sanctuary site for hematological malignancies due to the presence of the testicular-blood barrier. Hence, it is imperative to keep a high index of suspicion for testicular plasmacytoma in the right clinical context when evaluating a patient with known multiple myeloma.

Polyserositis as a Unique Presentation of Isolated Myeloid Sarcoma: A Case Report.

BACKGROUND: Myeloid Sarcoma (MS) are tumors containing myeloid blasts occurring in a location other than the bone marrow, including lymph nodes, skin, and soft tissues. MS presenting as polyserositis however is very rare, with only a few cases in the literature. CASE REPORT: A 20-year-old male presented with cough, shortness of breath and was found to have left upper lobe consolidation, left pleural effusion, pericardial effusion, and a large anterior mediastinal mass. A transthoracic echocardiogram showed pericardial effusion with tamponade physiology. He underwent emergent pericardiocentesis and thoracentesis. The fluid studies showed flow cytometry findings consistent with MS/ acute myeloid leukemia (AML) phenotype. A bone marrow aspirate and biopsy were unremarkable and showed no immunophenotypic findings diagnostic of acute leukemia or a lymphoproliferative disorder. Cytogenetics was negative for AML abnormalities per FISH analysis. Videoassisted thoracoscopy surgery (VATS) with biopsy of the mediastinal mass, pericardium, and left upper lobe of the lung was consistent with MS. He was treated with induction cytarabine and idarubicin, and a follow up PET-CT scan showed complete remission. He is currently day 200 + post stem cell transplant with no evidence of disease recurrence. CONCLUSION: To the best of our knowledge, this is the first case of isolated myeloid sarcoma presenting as polyserositis, without prior leukemia/ bone marrow involvement. Hence, fluid studies should involve cytometry analysis and MS should be entertained as a differential for polyserositis, even without a history of prior leukemia. Timely diagnosis can expedite aggressive chemotherapy required for a potentially life-threatening disease.

Patterns of treatment and their outcomes in primary breast lymphoma; a comprehensive population-based analysis.

BACKGROUND: Primary breast lymphoma (PBL) is managed differently among centers, using surgery, systemic therapy and/or radiation. With data derived from the National Cancer Database (NCDB), we aim to describe treatments utilized in the United States, estimate the overall survival (OS) of different therapeutic modalities and determine the role of systemic therapy in patients with PBL. METHODS: We conducted a retrospective cohort study using de-identified data from the NCDB. The NCDB provided records of 4616 patients diagnosed with PBL between 2004 and 2015. We excluded patients diagnosed with HIV, with no survival data, not treated in the reporting facility, without histologic confirmation, with stage III/ IV disease and for whom surgery, radiation, or systemic therapy was contraindicated. Both propensity score weighting and Cox models were used to obtain adjusted estimates. Based on histopathology, PBL was classified into indolent (I-PBL) and aggressive (A-PBL). RESULTS: In a sample size of 2063 PBL patients, the median age was 67 years (interquartile range (IQR): 57-78), and 97% were females. In 1027 patients with I-PBL, the median follow-up was 66 months (95% confidence interval (CI): 32.6-107.2) and 60% of patients had extranodal marginal zone subtype. Systemic therapy did not improve adjusted-OS (median: 154 vs. 143 months, P = 0.36) (Hazard ratio (HR): 0.86, 95% CI: 0.60-1.25, P = 0.42). The treatment arms associated with the highest adjusted 5-year OS were as follows: radiation (85%), surgery (79%), systemic & radiation (87%) and radiation & surgery (87%) (P = 0.9). In 1036 patients with A-PBL, the median follow-up was 67.4 months (95% CI: 35.9-105), and 87% of patients had diffuse large B-cell subtype. Patients with A-PBL who received systemic therapy had an improved adjusted-OS (median: 115 vs. 72 months, P < 0.01) (HR: 0.45, 95% CI: 0.38-0.53, P < 0.001). The treatment arms associated with the highest adjusted 5-year OS were: systemic (69%), systemic & radiation (77%), systemic & radiation & surgery (79%) and systemic & surgery (79%) (P = 0.4). CONCLUSIONS: Systemic therapy used as first-line treatment is essential in A-PBL. Local therapy in the I-PBL using surgery and/or radiation is effective in long-term disease control. There is significant variation in front-line treatment modalities utilized in PBL across the US, many associated with similar outcomes.

Intrathecal Chemotherapy as a Potential Treatment for Steroid-refractory Immune Effector Cell-associated Neurotoxicity Syndrome.

BACKGROUND/AIM: Chimeric antigen receptor (CAR) T-cell therapy has revolutionized the treatment of various B-cell malignancies. However, it can cause serious adverse effects like immune effector cell-associated neurotoxicity syndrome (ICANS). ICANS is attributed to disruption of the blood-brain barrier due to inflammatory cytokines and increased levels of immune effector cells (IECs) in the cerebrospinal fluid (CSF). Corticosteroids and supportive management are the mainstays of ICANS treatment. However, no guidelines exist for the treatment of steroid-refractory ICANS. Some reports have shown favorable outcomes with no long-term complications in patients with steroid-refractory ICANS treated with intrathecal (IT) chemotherapy. CASE REPORT: We describe the outcomes of two patients with steroid-refractory ICANS treated with IT chemotherapy. Both patients had refractory large B-cell lymphoma and were not candidates for autologous transplant. They developed steroid-refractory ICANS after CAR T-cell infusion. IT chemotherapy with 12 mg methotrexate and 50 mg hydrocortisone resulted in prompt neurological improvement in both patients. One of them passed away due to multiple other comorbidities, and the other patient continues to do well without any complications. CONCLUSION: IT chemotherapy could be considered as a potential approach for the management of steroid-refractory ICANS based on our experience. Prospective studies are needed to validate this approach.

Consolidative Autologous Stem Cell Transplantation Versus Whole Brain Radiation in PCNSL; a Nationwide Analysis.

BACKGROUND: The best consolidation strategy after induction chemotherapy in Primary CNS Lymphoma (PCNSL) remains controversial. Our objective is to estimate the overall survival (OS) for autologous stem cell transplantation (ASCT) versus whole brain radiation (WBRT) in the consolidation setting. We also sought to evaluate the factors affecting treatment selection METHODS: We identified 1620 patients with PCNSL who received chemotherapy followed by either ASCT or WBRT between 2004 and 2015 from the National Cancer Database. A propensity score weighting methodology was used to compare survival outcomes. Factors affecting treatment selection were investigated using a logistic regression model. Annual percentage change (APC) was calculated to assess the trend of ASCT use. RESULTS: Only 12.2% of patients received ASCT, and this proportion rose steadily between 2004 and 2015, with APC of +23%. Treatment selection was affected by age, type of area, distance from the treating facility, and level of education. With a median follow-up of 68.4 months, adjusted-median OS was 91.4 months and not reached for WBRT and ASCT groups, respectively (P < .001). 5-year OS was 74.4% in the ASCT group versus 58.7% in the WBRT group (HR 0.40, 95% CI 0.27-0.60, P -value < .01). CONCLUSION: Socioeconomic factors affect the selection of consolidative treatment in patients with PCNSL which can alter outcomes. Frequency of consolidative ASCT is increasing for patients with PCNSL. This is the first and largest cohort study, to our knowledge, to show an OS advantage in favor of ASCT. This OS benefit needs to be confirmed in a randomized controlled fashion.

A Case of Systemic AL Amyloidosis Diagnosed by Screening Colonoscopy.

Amyloidosis encompasses several diseases associated with deposition of low-molecular-weight proteins in an abnormal configuration. In light-chain amyloidosis (AL), monoclonal free lambda (λ) or kappa (κ) light chains are the amyloid proteins involved and can deposit in almost any organ. Symptoms vary depending on presence and extent of organ involvement, and thus, clinical presentation varies. Diagnosis requires biopsy of the affected tissue, and sometimes, fat pad or bone marrow biopsy is completed initially. Prognosis of AL amyloidosis depends on the presence of cardiac involvement. Treatment of AL amyloidosis involves systemic chemotherapy and evaluation for autologous stem cell transplant. Herein, we present a case report of an asymptomatic middle-aged female who was diagnosed with AL amyloidosis during an average-risk screening colonoscopy, which is an unusual setting. We discuss the workup involved, clinical presentation, and gastroenterology-related organ involvement.

Predictors and Long-Term Outcomes for Diffuse Large B-Cell Lymphoma (DLBCL) Patients Undergoing Surgery Prior to Systemic Therapy: A Nationwide Analysis.

BACKGROUND: A minority of patients diagnosed with diffuse large B-cell lymphoma (DLBCL) undergo surgery before the initiation of systemic therapy. The aim of this study is to explore the characteristics of patients undergoing surgery prior to systemic therapy (surgfirst), the predictors for surgfirst, and the survival outcomes. METHODS: The National Cancer Database was queried for patients with DLBCL diagnosed between 2006 and 2015, and we performed a subgroup analysis of patients that received surgfirst. Time-to-initial therapy (TTI) was defined as the time in days (d) from diagnosis to systemic therapy. Overall survival was measured from the day of diagnosis in terms of months (m). RESULTS: Factors associated with lower likelihood of surgfirst were non-Hispanic Black race (p-value<0.005), rural location (p-value<0.005), treatment at academic center (p-value<0.005), Medicaid insurance (p-value=0.01), comorbidity score >=3 (p-value 0.007), year of diagnosis, advanced stages of disease, and presence of B-symptoms. The TTI of systemic therapy was delayed in the surgfirst group - 34 (IQR 22-52) days vs. 23 (IQR 13-38) days, p-value<0.005. The five-year overall survival was 62.7% (95% CI 62.1-63.2%) vs. 58.3% (95% CI 57.7-60.0%) - HR 0.87 (95% CI 0.85-0.89), p-value<0.005. The factors associated with higher mortality were advanced comorbidities, lower educational status, disease primarily located in the bone, brain, and spinal cord, advanced clinical stage, presence of B-symptoms, and advanced age. CONCLUSION: Despite the delay in systemic therapy, we could not identify a detrimental impact of surgfirst on survival. This needs to be confirmed in large-scale multicenter studies. We identified clinical and socioeconomic factors that affect treatment selection and survival.

The Trend of Combined Modality Treatment and its Outcomes in Elderly Patients With Primary CNS Lymphoma: A 12-year Population-based Analysis Using Propensity Score.

BACKGROUND/AIM: The addition of radiation to chemotherapy in elderly patients with primary central nervous system lymphoma (PCNSL) remains controversial. This aim of this study was to assess the trend of combined modality treatment (CMT) and compare its survival with chemotherapy alone and radiation alone in non-HIV patients. PATIENTS AND METHODS: We identified 6,537 patients who received single treatment modality, CMT, or no treatment at all between 2004 and 2015 from the National Cancer Database. Factors affecting treatment selection were investigated using a logistic regression model. Annual percentage change (APC) was calculated to assess the trend of CMT use. A propensity score weighting methodology was used to compare survival outcomes. RESULTS: Only 12.8% of patients received CMT, and this proportion steadily declined between 2004 (17.7%) and 2015 (8.7%), with an APC of -6.0% (95%CI=-8.0 - -4.0, p-value <0.001) during the 12 years. Apart from classical prognostic factors (age and comorbidities), treatment selection was significantly influenced by sex, facility type, degree of urbanization, and type of insurance. CMT had improved survival [median overall survival 19.5 months (95%CI=15.7-22.8)] compared with single-modality treatment. This effect was more prominent in the first year. CONCLUSION: Socioeconomic factors affect the selection of treatment in elderly patients with PCNSL. CMT is falling out of favor in this patient population due to the risks of neurotoxicity. Further work should focus on developing strategies that minimize toxicity and access disparities without compromising survival.

Three Cases of Lenalidomide Therapy for Multiple Myeloma and Subsequent Development of Secondary B-ALL.

INTRODUCTION: Multiple myeloma (MM) is the second most common hematological malignancy, accounting for 1% of all cancers, with median age of diagnosis between 66-70 years. MM remains incurable despite advances in treatment over time. Lenalidomide is an important medication used in induction therapy for MM and is also used for maintenance therapy for standard risk patients. With its increasing use, data is emerging about its use being associated with increased risk of secondary primary malignancies (SPM), especially when used as maintenance therapy. CASE SERIES: In this case series, we describe three patients with refractory MM treated with lenalidomide maintenance who later developed sALL. All had a common presentation of pancytopenia. They developed cytopenias while being on lenalidomide which was refractory to lenalidomide cessation, prompting bone marrow biopsy. MANAGEMENT AND OUTCOME: Lenalidomide was subsequently stopped, and patients were treated for secondary B-ALL. However, all passed away either due to relapse of disease or complications arising from treatment. DISCUSSION: The mechanism of lenalidomide associated SPMs is not well understood however its incidence is well documented. At least 13 cases of ALL (predominantly B-cell ALL) following Immunomodulator imide drugs (IMiDs) have been reported in literature. An analysis of a larger cohort of patients is required to determine causality of lenalidomide with sALL. However, benefits of maintenance lenalidomide in patients with MM outweighs the risk of developing SPMs. Albeit persistent pancytopenia on lenalidomide therapy should be evaluated with bone marrow biopsy since it could be caused by secondary B -cell ALL.

Management of congenital dysfibrinogenemia in pregnancy: A challenging patient case.

Afibrinogenemia and congenital dysfibrinogenemia (CD) are rare conditions with limited information available for appropriate management. Previous case reports have demonstrated the safe and efficacious use of fibrinogen replacement therapy (FRT) as a therapeutic approach to prevent hemorrhage and fetal loss in pregnant women with CD. In this case report, we present a 28-year-old pregnant woman who sought testing for CD given her family history. She denied any current or previous bleeding symptoms. Laboratory testing confirmed the diagnosis of CD. She was treated with FRT and prophylactic anticoagulation starting in her third trimester. She had preterm labor that prompted an urgent cesarean section with FRT support. This case adds to the sparse literature about fibrinogen disorders in pregnancy, and highlights the benefits, safety, and tolerability of FRT and prophylactic anticoagulation in pregnant women with CD. Finally, it emphasizes the importance of a multidisciplinary team approach for an uneventful delivery.