Non-Hodgkin's Lymphoma Associated with Sickle Cell Disease: A Case Report

1987 ◽  
Vol 73 (5) ◽  
pp. 523-524 ◽  
Author(s):  
Fortunato Morabito ◽  
Vincenzo Callea ◽  
Maura Brugiatelli ◽  
Domenico D'Ascola ◽  
Alfio Palazzolo ◽  
...  

A case of non-Hodgkin's lymphoma with leukemic spread in a patient affected with homozygous sickle cell disease is reported. This association has not been previously described. A correlation between the malignancy and the hemoglobinopathy could not be etiologically ascertained; therefore, an alternative explanation to a chance event cannot be offered.

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4872-4872 ◽  
Author(s):  
Ahmar Urooj Zaidi ◽  
Lo'Rell Martin ◽  
Michael U Callaghan ◽  
Sharada A. Sarnaik ◽  
Jeffrey W Taub

Abstract Background: Sickle cell disease affects 100,000 people in the United States. Cancer does not appear to be more common in individuals with sickle cell disease however many presenting signs and symptoms of cancer overlap with signs and symptoms of sickle cell disease, complicating diagnosis. Furthermore, sickle cell disease can complicate surgical, chemo- and radio- therapies for malignancy. Methods: We reviewed the past 46 years experience of cancers in children with sickle cell at a large urban pediatric referral center. We identified 5 cases and reviewed the presentations, treatments, response to therapy, sickle cell complication and laboratory values, including transfusion needs and effect of chemotherapy on fetal hemoglobin levels. Results: We identified five cases of malignancies in children with sickle cell disease including cases of acute lymphoblastic leukemia (ALL), Hodgkin's lymphoma, Non-Hodgkin's lymphoma (NHL), Wilms tumor and renal cell carcinoma. An additional patient with sickle cell trait was diagnosed with metastatic renal medullary carcinoma. The patients were ages 6-18 years old at diagnosis and 4 were female. All had delays in diagnosis as their initial signs and symptoms overlapped with sickle cell disease (abdominal mass mimicking splenomegaly, low blood counts, fever and posterior reversible encephalopathy syndrome, hematuria). All five achieved complete remission with multi-modality therapy (excluding stem cell transplants) and are currently doing well 1- 20 years after treatment. Admissions for pain were reduced during and for some period after cancer therapy and 2 patients had marked, and in one case, prolonged elevation of fetal hemoglobin after therapy. Discussion: Children with sickle cell develop cancers that can be confused for sickle cell related complications. Cancer therapy can be well tolerated and successful in children with sickle cell. Sickle cell complications are decreased during cancer therapy likely secondary to frequent therapy related transfusion and therapy induced increases in fetal hemoglobin. Disclosures Callaghan: Biogen: Honoraria; Bayer: Honoraria; CSL Behring: Honoraria; Baxalta: Honoraria, Research Funding; Grifols: Honoraria; Roche: Honoraria, Research Funding.


2018 ◽  
Vol 40 (2) ◽  
pp. 118-120 ◽  
Author(s):  
Zainab Abdulmajeed Toorani ◽  
Srishma Sridhar ◽  
Wilfredo Roque

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5889-5889
Author(s):  
Stuthi Pavani Perimbeti ◽  
Kevin Ye Hou ◽  
Sabarina Ramanathan ◽  
Adonas Woodard ◽  
Daniel Kyung ◽  
...  

Abstract Introduction: Case reports have suggested that there is an increased risk of hematological malignancies with sickle cell disease (SCD). We aimed to investigate the prevalence and mortality of select hematological malignancies, including: acute myeloid leukemia (AML), acute lymphoblastic leukemia (ALL), polycythemia vera (PV), essential thrombocytopenia (ET), Hodgkin's lymphoma (HL) and non-Hodgkin's lymphoma (NHL), primary myelofibrosis (PMF) in patients hospitalized with SCD. Methods: We queried the Healthcare Cost and Utilization Project's Nationwide Inpatient Sample to identify the hospitalizations with SCD using ICD-9 codes 282.6, 282.60, 282.61, 282.62, 282.63, 282.64, 282.68, 282.69 from 1999 to 2014. Cases were then stratified based on the concurrent presence of the above hematological malignancies. The percentages of patients with each type of hematological malignancy were obtained using Chi-square analysis. In addition, we compared outcomes between patients with and without SCD who had hematological malignancies. Bivariate analysis for in-hospital mortality percentage was performed using Chi-square test. Multivariate analysis to evaluate the risk of death during hospitalization was performed using Cox proportional hazard regression with alpha set at 0.05. Results: There were 307,424 admissions (weighted=1,513,168) with SCD. Within these admissions, 0.04% (n=516) were associated with AML, 0.05% (n=720) with HL, 0.02% (n=47) with ALL, 0.07% (n=1,028) with NHL, 0.69% (n=10,654) with ET, 0.01% (n=20) with PMF and 0.01% (n=18) with PV. The hazard ratio for mortality (95% C.I.) with SCD compared to without SCD was 3.60 (1.24-4.67) for AML (p <0.001), 4.56 (2.78-6.78) for ET (p <0.001), 2.37 (1.41-5.67) for NHL (p=0.003), 1.5 (0.8-4.5) for HL (p=0.08), 1.1 (0.5-3.2) for ALL (p=0.04), 1.3 (1.1-3.2) for PMF (p= 0.03) and 1.7 (0.6-2.9) for PV (p=0.05). Discussion: The most frequently encountered hematological malignancy in patients with SCD was ET, followed by NHL and then HL. After controlling for multiple confounders including age, race, sex, comorbidities and socioeconomic status, the hazard of death during hospitalization with SCD, ET, AML, NHL and ALL are significantly higher compared to those without SCD. While uncommonly encountered, concomitant hematological malignancy and SCD portends a significantly worse outcome, particularly in ET and AML. Potential explanations include iron overload from prior transfusions, increased infection risk due to asplenia and vascular damage from previous vaso-occlusive events. Newer advances in the management of SCD might improve subsequent outcomes in hematological malignancies. Disclosures No relevant conflicts of interest to declare.


1993 ◽  
Vol 70 (04) ◽  
pp. 568-572 ◽  
Author(s):  
Roberto Stasi ◽  
Elisa Stipa ◽  
Mario Masi ◽  
Felicia Oliva ◽  
Alessandro Sciarra ◽  
...  

SummaryThis study was designed to explore the prevalence and clinical significance of elevated antiphospholipid antibodies (APA) titres in patients affected by acute myeloid leukemia (AML) and highgrade non-Hodgkin’s lymphoma (NHL). We also analyzed possible correlations with circulating levels of interleukin-6 (IL-6), tumor necrosis factor-alpha (TNF-alpha), and the soluble form of the receptor for interleukin-2 (sIL-2r). Nineteen patients with de novo AML and 14 patients with newly-diagnosed NHL were investigated. Tests for APA included the measurement of anticardiolipin antibodies (ACA) with a solid-phase immunoassay, and the detection of the lupus-like anticoagulant (LA) activity. Five patients with AML (26.3%) and 5 patients with NHL (35.7%) presented elevated APA at diagnosis, as compared to 3 of 174 persons of the control group (p <0.0001). APA titres became normal in all patients responding to treatment, whereas nonresponders retained elevated levels. In addition, 6 patients (4 with AML and 2 with NHL), who had normal APA at diagnosis and were either refractory to treatment or in relapse, subsequently developed LA and/or ACA positivity. At presentation, the mean levels of IgG- and IgM-ACA in patients were not significantly different from Controls, and concordance between ACA and LA results reached just 30%. With regard to the clinical course, we were not able to detect any statistically significant difference between patients with normal and elevated APA. Pretreatment concentrations of IL-6 and TNF-alpha in AML, and sIL-2r in NHL were found significantly elevated compared to Controls (p = 0.003, p = 0.009 and p = 0.024 respectively). In addition, the levels of these cytokines correlated with IgG-ACA at the different times of laboratory investigations. These results demonstrate that APA may have a role as markers of disease activity and progression in some haematological malignancies.


1996 ◽  
Vol 76 (03) ◽  
pp. 322-327 ◽  
Author(s):  
Dominique Helley ◽  
Amiram Eldor ◽  
Robert Girot ◽  
Rolande Ducrocq ◽  
Marie-Claude Guillin ◽  
...  

SummaryIt has recently been proved that, in vitro, red blood cells (RBCs) from patients with homozygous β-thalassemia behave as procoagulant cells. The procoagulant activity of β-thalassemia RBCs might be the result of an increased exposure of procoagulant phospholipids (i. e. phosphatidylserine) in the outer leaflet of the membrane. In order to test this hypothesis, we compared the catalytic properties of RBCs of patients with β-thalassemia and homozygous sickle cell disease (SS-RBCs) with that of controls. The catalytic parameters (Km, kcat) of prothrombin activation by factor Xa were determined both in the absence and in the presence of RBCs. The turn-over number (kcat) of the reaction was not modified by normal, SS- or (3-thalassemia RBCs. The Km was lower in the presence of normal RBCs (mean value: 9.1 µM) than in the absence of cells (26 µM). The Km measured in the presence of either SS-RBCs (mean value: 1.6 µM) or β-thalassemia RBCs (mean value: 1.5 pM) was significantly lower compared to normal RBCs (p <0.001). No significant difference was observed between SS-RBCs and p-thalassemia RBCs. Annexin V, a protein with high affinity and specificity for anionic phospholipids, inhibited the procoagulant activity of both SS-RBCs and (3-thalassemia RBCs, in a dose-dependent manner. More than 95% inhibition was achieved at nanomolar concentrations of annexin V. These results indicate that the procoagulant activity of both β-thalassemia RBCs and SS-RBCs may be fully ascribed to an abnormal exposure of phosphatidylserine at the outer surface of the red cells.


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