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1.
Am J Hematol ; 99(6): 1205-1207, 2024 Jun.
Article in English | MEDLINE | ID: mdl-38602288

ABSTRACT

The findings of this study highlight a 95% accuracy rate in ChatGPT responses, as assessed by five myeloma specialists, underscoring its potential as a reliable educational tool.


Subject(s)
Multiple Myeloma , Patient Education as Topic , Multiple Myeloma/therapy , Multiple Myeloma/diagnosis , Humans , Male , Female
3.
BMJ Case Rep ; 16(12)2023 Dec 28.
Article in English | MEDLINE | ID: mdl-38154873

ABSTRACT

Composite lymphoma is defined as two or more lymphomas with distinct morphological and immunophenotypical characteristics synchronously diagnosed at the same anatomical site. Composite lymphoma is rare, and the most common combination is follicular lymphoma (FL) associated with diffuse large B cell lymphoma, followed by FL associated with classic Hodgkin's lymphoma (HL). Histologically, composite lymphomas display a mixed pattern or distinct zonal distribution of each lymphoma component. Composite lymphoma poses a diagnostic challenge, especially when two lymphoma components are mixed in the same lymph node. Here, we report a case of composite HL and FL 11 years after initial and repeat biopsies consistent with FL in a man in his 70s emphasising the importance of repeat biopsy in lymphoma diagnosis.


Subject(s)
Composite Lymphoma , Hodgkin Disease , Lymphoma, Follicular , Lymphoma, Large B-Cell, Diffuse , Male , Humans , Lymphoma, Follicular/diagnosis , Lymphoma, Follicular/pathology , Hodgkin Disease/diagnosis , Hodgkin Disease/pathology , Composite Lymphoma/diagnosis , Neoplasm Recurrence, Local , Lymphoma, Large B-Cell, Diffuse/pathology
4.
J Hematol ; 10(1): 25-29, 2021 Feb.
Article in English | MEDLINE | ID: mdl-33643507

ABSTRACT

Waldenstrom's macroglobulinemia (WM) is an indolent B-cell non-Hodgkin lymphoma characterized by lymphoplasmacytic histology in the bone marrow with monoclonal IgM. Median survival can be in excess of 10 years. The 5-year cumulative incidence of death is low at about 10%. One-third of all-cause specific mortality is due to the lymphoma for which histologic transformation (HT) is rare. Here we present a case of a 60-year-old man with longstanding untreated WM, presenting with minimally symptomatic transformation to diffuse large B-cell lymphoma (DLBCL), with an accompanying review of the literature. Transformed WM, diagnosed greater than 5 years, has a reported survival period of 8 - 9 months. This case highlights that after a decade of continued stability in WM, not requiring treatment, an acute change in laboratory data with minimally progressive IgM levels, in the absence of B symptoms and clinical findings, may be the harbinger of transformation and at the time of diagnosis can have a rapidly deteriorating clinical course. In this case, the tripling of the lactate dehydrogenase (LDH) as the primary drastic change demonstrates the importance of the rapid increase in LDH as a singly reliable marker for HT. Late transformation has been borne out as a negative variable as the generally indolent course of WM is curtailed with the poor outcome in HT. Although MYD88 wildtype is a possible predictive factor for transformation, it is unclear if late transformation is clonally or non-clonally related and further molecular investigation is needed.

5.
Clin Med Insights Blood Disord ; 14: 2634853521999389, 2021.
Article in English | MEDLINE | ID: mdl-33716516

ABSTRACT

Primary plasma cell leukemia (PCL) is a rare and aggressive variant of multiple myeloma (MM). PCL is characterized by peripheral blood involvement by malignant plasma cells and an aggressive clinical course leading to poor survival. There is considerable overlap between MM and PCL with respect to clinical, immunophenotypic, and cytogenetic features, but circulating plasma cell count exceeding 20% of peripheral blood leukocytes or an absolute plasma cell count of >2000/mm3 distinguishes it from MM. After initial stabilization and diagnosis confirmation, treatment of PCL in a fit patient typically includes induction combination chemotherapy containing novel agents typically, with proteasome inhibitors (such as bortezomib) and immunomodulatory drugs (eg, lenalidomide), followed by autologous hematopoietic stem cell transplant (HSCT) and multidrug maintenance therapy using novel agents post-HSCT. Long-term outcomes have improved employing this strategy but the prognosis for non-HSCT candidates remains poor and new approaches are needed for such PCL patients not eligible for HSCT. Here, we report a case of primary PCL, and a comprehensive and up to date review of the literature for diagnosis and management of PCL. We also present the findings of Positron Emission Tomography (PET) scan. Since PCL is often associated with extra-medulary disease, including PET scan at the time of staging and restaging may be a novel approach particularly to evaluate the extra-medullary disease sites.

7.
Cardiooncology ; 6: 9, 2020.
Article in English | MEDLINE | ID: mdl-32690995

ABSTRACT

BACKGROUND: Cardio-Oncology (CO) is a new subspecialty that thrives mostly in large academic quaternary centers. This study describes how to establish a successful cardio-oncology program, with limited resources, in order to effectively manage the unique care required by this patient population. METHODS: Clinical data was collected from 25 consecutive months. There were four foundational elements to establish a CO program: 1. Clinical program: integrating staff and resources from the Heart and Vascular, and Cancer Centers; 2. Education Program: establishing a platform to educate/advocate with respect to CO; 3. Engagement with professional societies: active engagement allowed for the successful establishment of the proposed CO program; and 4. Research program: establishing data collection modalities/cooperation with other institutions. RESULTS: 474 consecutive patients were treated by our CO program during the first 25 months of operation. Clinical data, information about cancer treatment, cardiovascular co morbidities, cardiac testing and impact of CO management are reported. CONCLUSIONS: A successful CO program can be established utilizing existing resources without the need for significant additional assets. Integration with professional societies, advocacy, education and research, provide a platform for learning and growth. This model improves access to care and can be reproduced in a variety of settings.

9.
Immunotherapy ; 11(2): 81-89, 2019 02.
Article in English | MEDLINE | ID: mdl-30499734

ABSTRACT

AIM: To evaluate the safety and efficacy of 10% intravenous immunoglobulin (IVIG; Flebogamma® 10% DIF) in individuals with chronic immune thrombocytopenic purpura (ITP). PATIENTS & METHODS: Patients aged 3-70 years, diagnosed with chronic ITP, received 1 g/kg IVIG over two consecutive days. RESULTS: 64 evaluable patients (51 adults, 13 children) with chronic ITP received IVIG. The primary efficacy end point (increased platelet counts from ≤20 × 109/l to ≥50 × 109/l by day 8) was achieved by 81.3% of patients; mean time to response was 1.7 days (all responders). Adverse events, mostly mild or moderate, were reported in 59 patients (92.2%). CONCLUSION: Flebogamma® 10% DIF administered over two consecutive days was safe and effective in adults and children with chronic ITP.


Subject(s)
Blood Platelets/pathology , Immunoglobulins, Intravenous/therapeutic use , Purpura, Thrombocytopenic, Idiopathic/drug therapy , Adolescent , Adult , Aged , Child , Child, Preschool , Female , Humans , Leukocyte Count , Male , Middle Aged , Treatment Outcome , Young Adult
10.
Am J Hematol ; 85(8): 553-9, 2010 Aug.
Article in English | MEDLINE | ID: mdl-20658586

ABSTRACT

Paroxysmal nocturnal hemoglobinuria (PNH) is a debilitating and life-threatening disease in which lysis of PNH red blood cells frequently manifests with chronic hemolysis, anemia, and thrombosis. Renal damage in PNH is associated with chronic hemosiderosis and/or microvascular thrombosis. We determined the incidence of renal dysfunction or damage, defined by stages of chronic kidney disease (CKD), in a large cohort of PNH patients and evaluated the safety and efficacy of the complement inhibitor eculizumab in altering its progression. Renal dysfunction or damage was observed in 65% of the study population at baseline with 21% of patients with later stage CKD or kidney failure (glomerular filtration rate [GFR]

Subject(s)
Antibodies, Monoclonal/therapeutic use , Complement C5/antagonists & inhibitors , Complement Inactivating Agents/therapeutic use , Hemoglobinuria, Paroxysmal/drug therapy , Kidney Failure, Chronic/prevention & control , Kidney/physiopathology , Adolescent , Adult , Aged , Aged, 80 and over , Antibodies, Monoclonal/pharmacology , Antibodies, Monoclonal, Humanized , Complement Activation/drug effects , Complement C5/immunology , Female , Glomerular Filtration Rate/drug effects , Hemoglobinuria, Paroxysmal/complications , Hemoglobinuria, Paroxysmal/immunology , Hemoglobinuria, Paroxysmal/physiopathology , Hemolysis/drug effects , Humans , Kidney/drug effects , Kidney Failure, Chronic/drug therapy , Kidney Failure, Chronic/etiology , Kidney Failure, Chronic/physiopathology , Male , Metabolic Clearance Rate/drug effects , Middle Aged , Pilot Projects , Severity of Illness Index , Treatment Outcome , Young Adult
11.
Blood ; 111(4): 1840-7, 2008 Feb 15.
Article in English | MEDLINE | ID: mdl-18055865

ABSTRACT

The terminal complement inhibitor eculizumab was recently shown to be effective and well tolerated in patients with paroxysmal nocturnal hemoglobinuria (PNH). Here, we extended these observations with results from an open-label, non-placebo-controlled, 52-week, phase 3 clinical safety and efficacy study evaluating eculizumab in a broader PNH patient population. Eculizumab was administered by intravenous infusion at 600 mg every 7 +/- 2 days for 4 weeks; 900 mg 7 +/- 2 days later; followed by 900 mg every 14 +/- 2 days for a total treatment period of 52 weeks. Ninety-seven patients at 33 international sites were enrolled. Patients treated with eculizumab responded with an 87% reduction in hemolysis, as measured by lactate dehydrogenase levels (P < .001). Baseline fatigue scores in the FACIT-Fatigue instrument improved by 12.2 +/- 1.1 points (P < .001). Eculizumab treatment led to an improvement in anemia. The increase in hemoglobin level occurred despite a reduction in transfusion requirements from a median of 8.0 units of packed red cells per patient before treatment to 0.0 units per patient during the study (P < .001). Overall, transfusions were reduced 52% from a mean of 12.3 to 5.9 units of packed red cells per patient. Forty-nine patients (51%) achieved transfusion independence for the entire 52-week period. Improvements in hemolysis, fatigue, and transfusion requirements with eculizumab were independent of baseline levels of hemolysis and degree of thrombocytopenia. Quality of life measures were also broadly improved with eculizumab treatment. This study demonstrates that the beneficial effects of eculizumab treatment in patients with PNH are applicable to a broader population of PNH patients than previously studied. This trial is registered at http://clinicaltrials.gov as NCT00130000.


Subject(s)
Antibodies, Monoclonal/therapeutic use , Hemoglobinuria, Paroxysmal/drug therapy , Adolescent , Adult , Aged , Antibodies, Monoclonal/administration & dosage , Antibodies, Monoclonal/adverse effects , Antibodies, Monoclonal/pharmacokinetics , Antibodies, Monoclonal, Humanized , Area Under Curve , Blood Transfusion , Fatigue/chemically induced , Female , Hemoglobinuria, Paroxysmal/blood , Hemolysis/drug effects , Humans , Infusions, Intravenous , Male , Middle Aged , Placebos , Safety
12.
N Engl J Med ; 355(12): 1233-43, 2006 Sep 21.
Article in English | MEDLINE | ID: mdl-16990386

ABSTRACT

BACKGROUND: We tested the safety and efficacy of eculizumab, a humanized monoclonal antibody against terminal complement protein C5 that inhibits terminal complement activation, in patients with paroxysmal nocturnal hemoglobinuria (PNH). METHODS: We conducted a double-blind, randomized, placebo-controlled, multicenter, phase 3 trial. Patients received either placebo or eculizumab intravenously; eculizumab was given at a dose of 600 mg weekly for 4 weeks, followed 1 week later by a 900-mg dose and then 900 mg every other week through week 26. The two primary end points were the stabilization of hemoglobin levels and the number of units of packed red cells transfused. Biochemical indicators of intravascular hemolysis and the patients' quality of life were also assessed. RESULTS: Eighty-seven patients underwent randomization. Stabilization of hemoglobin levels in the absence of transfusions was achieved in 49% (21 of 43) of the patients assigned to eculizumab and none (0 of 44) of those assigned to placebo (P<0.001). During the study, a median of 0 units of packed red cells was administered in the eculizumab group, as compared with 10 units in the placebo group (P<0.001). Eculizumab reduced intravascular hemolysis, as shown by the 85.8% lower median area under the curve for lactate dehydrogenase plotted against time (in days) in the eculizumab group, as compared with the placebo group (58,587 vs. 411,822 U per liter; P<0.001). Clinically significant improvements were also found in the quality of life, as measured by scores on the Functional Assessment of Chronic Illness Therapy-Fatigue instrument (P<0.001) and the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire. Of the 87 patients, 4 in the eculizumab group and 9 in the placebo group had serious adverse events, none of which were considered to be treatment-related; all these patients recovered without sequelae. CONCLUSIONS: Eculizumab is an effective therapy for PNH.


Subject(s)
Antibodies, Monoclonal/therapeutic use , Complement C5/immunology , Hemoglobinuria, Paroxysmal/drug therapy , Adolescent , Adult , Aged , Aged, 80 and over , Antibodies, Monoclonal/adverse effects , Antibodies, Monoclonal, Humanized , Double-Blind Method , Erythrocyte Transfusion , Fatigue , Female , Hemoglobins/analysis , Hemoglobinuria, Paroxysmal/blood , Hemoglobinuria, Paroxysmal/therapy , Hemolysis/drug effects , Humans , Male , Middle Aged , Quality of Life
13.
J Lab Clin Med ; 139(4): 211-7, 2002 Apr.
Article in English | MEDLINE | ID: mdl-12024108

ABSTRACT

Histidine-rich glycoprotein (HRG) binds heparin and neutralizes its anticoagulant effect. Because zinc greatly enhances this interaction, it is possible that the combination of HRG and zinc could be used as an antidote for heparin. We have determined the plasma concentrations of HRG and zinc necessary to neutralize clinically relevant concentrations of heparin. Using a thrombin-time assay, we found that HRG plus zinc can neutralize 0.2 to 4.5 units/mL heparin, although the maximal effect requires an HRG plasma concentration about six times normal and a zinc concentration about 10 times normal. In a system using purified proteins and a thrombin-specific chromogenic substrate, we found that both the concentration of HRG and the molar ratio of HRG to zinc are important in determining the potency of the anti-heparin activity. Whether HRG and zinc can be administered as an antidote for heparin will depend on whether the requisite doses can be achieved without toxicity.


Subject(s)
Antidotes/pharmacology , Heparin Antagonists/pharmacology , Heparin/pharmacology , Proteins/pharmacology , Zinc/pharmacology , Antidotes/metabolism , Blood Coagulation/drug effects , Blood Proteins/metabolism , Blood Proteins/pharmacology , Citrates , Drug Synergism , Heparin/metabolism , Heparin Antagonists/metabolism , Humans , In Vitro Techniques , Protein Binding/drug effects , Proteins/metabolism , Thrombin Time , Zinc/metabolism
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