Acquired pure red cell aplasia is a rare disorder, usually appearing secondary to various pathologic conditions such as thymoma, systemic autoimmune diseases or in the course of lymphomas. Conventional treatment consists of immunosuppression with corticosteroids, antithymocyte globulin or cyclosporin-A. 8 weekly courses of rituximab were administered to a patient who presented with pure red cell aplasia secondary to newly diagnosed splenic marginal zone lymphoma. Transfusion independence was achieved after the 6th course, and pure red cell aplasia receded completely with therapy. Pure red cell aplasia may ensue early in the course of splenic marginal zone lymphoma and other low grade lymphomas. Rituximab is a safe and effective alternative treatment for pure red cell aplasia secondary to lymphoproliferative disorders.
Pure Red Blood Cell Aplasia - Etiology, pathophysiology, symptoms, signs, diagnosis & prognosis from the Merck Manuals - Medical Professional Version.
Anti-erythropoietin antibodies usually cross-react with all kinds of recombinant erythropoietins; therefore erythropoiesis-stimulating agent (ESA)-induced pure red-cell aplasia (PRCA) is not rescued by different ESAs. diagnosis. Interestingly anti-erythropoietin antibodies were still detectable although their concentration was too low for titration. In conclusion darbepoetin-α can improve ESA-induced PRCA when the anti-erythropoietin antibody titer declines and its neutralizing capacity is lost. Keywords: Red-Cell Aplasia Pure; Kidney Failure Chronic; Erythropoietin Recombinant; Darbepoetin-alfa INTRODUCTION Pure red-cell aplasia (PRCA) is a disorder of erythropoiesis that leads to sudden-onset progressive and severe anemia. Since 1998 there have been cases of recombinant human erythropoietin (rEPO) antibody-associated PRCA in patients with chronic kidney disease who receive subcutaneous treatment with rEPOs. In general patients developing erythropoiesis-stimulating agent (ESA)-induced PRCA ...
TY - JOUR. T1 - Isoniazid-induced pure red cell aplasia. AU - Holla, Subraya Krishna. AU - Achappa, Basavaprabhu. AU - Manibettu Raguram, Pavan. AU - Yerramsetti, Sitamahalakshminagasai. PY - 2018/1/1. Y1 - 2018/1/1. N2 - The pure red cell aplasia (PRCA) is an uncommon side effect of isoniazid. We describe a 28-year-old man who developed a severe anaemia caused by PRCA. The patient received antituberculous therapy including isoniazid for his pulmonary tuberculosis. On discontinuation of isoniazid, the anaemia recovered promptly. PRCA should be considered in case of unexplained anaemia during isoniazid treatment.. AB - The pure red cell aplasia (PRCA) is an uncommon side effect of isoniazid. We describe a 28-year-old man who developed a severe anaemia caused by PRCA. The patient received antituberculous therapy including isoniazid for his pulmonary tuberculosis. On discontinuation of isoniazid, the anaemia recovered promptly. PRCA should be considered in case of unexplained anaemia during ...
TY - JOUR. T1 - Zidovudine-induced reversible pure red cell aplasia. AU - Balakrishnan, Anuja. AU - Valsalan, Rohith. AU - Sheshadri, Shubha. AU - Pandit, Vinay R.. AU - Medep, Vikas. AU - Agrawal, Ravindra Kumar. PY - 2010/5/1. Y1 - 2010/5/1. N2 - Hematological abnormalities are frequent among human immunodeficiency virus (HIV)-infected patients and may be directly attributable to the virus or may be caused by opportunistic infections, neoplasms or drugs that cause bone marrow suppression or hemolysis. Pure red cell aplasia (PRCA) is an uncommon hematological disorder that causes anemia. We report a 37-year-old male with HIV infection who developed PRCA 6 weeks after commencing Zidovudine and recovered following cessation of the drug. This is the first case of Zidovudine-induced PRCA reported from the Indian subcontinent.. AB - Hematological abnormalities are frequent among human immunodeficiency virus (HIV)-infected patients and may be directly attributable to the virus or may be caused by ...
Pure red cell aplasia (PRCA) is a condition in which the bone marrow stops making red blood cells, causing anemia as well as reticulocytopenia, which is the absence of young red cells.
Also see Anemia: General Considerations.. Definition and EtiologyTop. Pure red cell aplasia (PRCA) refers to aplasia of the erythroid lineage leading to severe normocytic (in some cases macrocytic) and normochromic anemia. It may be inherited (Diamond-Blackfan syndrome) or acquired. In the latter case, it develops due to viral infections (parvovirus B19, Epstein-Barr virus, hepatotropic viruses), immunologic disorders (thymoma, myasthenia gravis, systemic lupus erythematosus, rheumatoid arthritis), chronic lymphocytic leukemia, treatment with an erythropoiesis-stimulating agent (ESA), or drugs (phenytoin, carbamazepine, valproate, azathioprine, chloramphenicol, sulfonamides, isoniazid). PRCA may precede myelodysplastic syndrome.. Clinical FeaturesTop. The diagnosis of PRCA is usually made in patients ,40 years. Clinical features include severe anemia with low reticulocyte counts, normal serum erythropoietin levels, and low bone marrow erythroblast counts (,0.5%). Clinical subtypes include acute ...
Pure Red Cell Aplasia in Major ABO-Mismatched Allogeneic Hematopoietic Stem Cell Transplantation Is Associated with Severe Pancytopenia.
Authors from the Versilia and Manzoni Hospitals in Italy report the case of a patient who developed pure red cell aplasia (PRCA) following subcutaneous
Diamond-Blackfan anemia (DBA) is a rare congenital disease caused by mutations in ribosomal protein genes and is characterized by pure red cell aplasia. While the prognosis is relatively favorable, quality of life (QOL) among DBA patients is negatively impacted by the adverse effects of long-term prednisolone (PSL) therapy and blood transfusions. We describe a 43-year-old man who was diagnosed with DBA (Hb of 2.18 g/dl) at the age of two months. He was initially treated with PSL and blood transfusions, followed by cyclosporine and low-dose (6 mg/day) PSL, which resulted in a sustained hemoglobin level of 9 g/dl without severe adverse events or loss of QOL ...
Acquired Pure Red Cell Aplasia information including symptoms, diagnosis, misdiagnosis, treatment, causes, patient stories, videos, forums, prevention, and prognosis.
Results He was noted to have anaemia requiring increasing doses of subcutaneous erythropoietin β up to 465 Units/kg/week for two years. Two months prior to transplantation, his haemoglobin was 4.6 g/dl with a low reticulocyte count of 2×109/l (0.1%) requiring his first blood transfusion of washed cells. His bone marrow aspirate and trephine biopsy one month before renal transplantation confirmed PRCA. There was no evidence of malignancy, haemolysis, haemorrhage, viral infection or auto-immune disease. His erythropoietin β was discontinued and he had high titres of anti epo Abs of 63U/ml (limit of positivity of 0.1 U/ml). He received his second blood transfusion before his renal transplant when his haemoglobin was 6.7 g/dl. He underwent a living related renal transplantation from his maternal uncle (mismatch 0,1,1) using triple immunosuppression (azathioprine and tacrolimus from one and two weeks prior to transplantation, respectively, with corticosteroids). His anti-epo Abs reduced further ...
Although the mechanism of post-transplant PRCA is not fully understood, it is believed that the persistence of host B lymphocytes or plasma cells producing anti-donor isoagglutinins is responsible for the delayed engraftment.12 In support of this hypothesis, autologous plasma derived from patients with PRCA inhibits donor-type erythropoiesis in vitro.18-20 In contrast, early erythroid progenitors can engraft at the same rate as myeloid progenitors measured by erythroid burst forming unit assays, indicating that ABO antigens are acquired at a later stage of erythroid commitment.21 Moreover, mixed chimerism analyses of hematopoietic cells after HSCT demonstrated temporal differences in the post-transplant eradication of recipient cells with a persistence of plasma cells for up to 9 months.12. In the present study the overall incidence of post-transplant PRCA was 8% and this complication occurred after major but not bidirectional ABO incompatible HSCT. The only two risk factors identified were a ...
The 5-year survivals for both groups were over 90% with no difference between responders and non-responders (Figure 4).. In this study, we expanded our original cohorts of patients with PRCA and mAA and included long-term follow-up for a median of 4.8 years (range 0.6-9.3 years). Daclizumab is effective in producing durable responses in patients with mAA and PRCA, without substantial acute or long-term toxicity. We chose to administer five infusions over the course of ten weeks with the option of a second course of therapy with relapse. However, it is unclear if the response rate would have been improved with more intensive therapy such as is utilized for uveitis.12 The majority of patients with both mAA and PRCA showed improvement in blood counts within three months of initiating therapy. Most responding patients with PRCA required two cycles of daclizumab to achieve sustained improvements in hemoglobin, but all had durable responses following their second course of treatment. The mAA patients ...
黴酚酸酯治療相關的常見藥物不良反應(大概出現於≥1%的患者)包括:腹瀉、噁心、嘔吐、關節痛;感染、白血球減少症,及/或反映藥物的免疫抑制和骨髓抑制性質的貧血。黴酚酸鈉通常也跟疲勞,頭痛,咳嗽和/或呼吸問題有關。黴酚酸酯的靜脈注射(IV)也通常與血栓形成和血栓性靜脈炎(英语:thrombophlebitis)有關。不尋常的副作用(佔0.1-1%的患者)包括食道炎(英语:esophagitis)、胃炎、消化道出血、及/或侵入性的巨細胞病毒(CMV)感染[10]。更罕見的是,肺部纖維化(英语:Pulmonary fibrosis)或各種贅生物的發生,如:黑色素瘤、淋巴瘤,其他惡性腫瘤的發生率則視乎類型而定,其發生的比例為1/20至1/200,而皮膚是腫瘤最常見的位置[21][22]。據報也有幾宗純紅細胞發育不全(PRCA)(英语:Pure red cell aplasia)的病例[23]。 ...
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Diamond-Blackfan anemia (DBA), a congenital bone-marrow-failure syndrome, is characterized by red blood cell aplasia, macrocytic anemia, clinical heterogeneity, and increased risk of malignancy. Although anemia is the most prominent feature of DBA, the disease is also characterized by growth retardation and congenital anomalies that are present in approximately 30%-50% of patients. The disease has been associated with mutations in four ribosomal protein (RP) genes, RPS19, RPS24, RPS17, and RPL35A, in about 30% of patients. However, the genetic basis of the remaining 70% of cases is still unknown. Here, we report the second known mutation in RPS17 and probable pathogenic mutations in three more RP genes, RPL5, RPL11, and RPS7. In addition, we identified rare variants of unknown significance in three other genes, RPL36, RPS15, and RPS27A. Remarkably, careful review of the clinical data showed that mutations in RPL5 are associated with multiple physical abnormalities, including craniofacial, thumb, and
What every physician needs to know: Pure red cell aplasia (PRCA) is defined as anemia with absent reticulocytes and marrow erythroid precursor cells. This rare aregenerative anemia has a number of interesting clinical associations and is also usually responsive to treatment. PRCA in children is very rare. Furthermore, the differential diagnosis is restricted predominantly to…. ...
Using this medicine may increase your risk of getting skin cancer or cancer of the lymph system (lymphoma). Talk to your doctor if you have concerns about this risk. Use sunscreen or sunblock lotions with a sun protection factor (SPF) of at least 15 on a regular basis when you are outdoors. Wear protective clothing and hats and stay out of direct sunlight, especially between the hours of 10 a.m. and 3 p.m. Avoid sunlamps and tanning beds. Mycophenolate can temporarily lower the number of white blood cells in your blood, which increases the chance of getting an infection. If you can, avoid people with infections. Check with your doctor immediately if you think you are getting an infection or if you have a fever or chills, cough or hoarseness, lower back or side pain, or painful or difficult urination. Mycophenolate may cause pure red cell aplasia (PRCA). This is a very rare condition where the body no longer makes red blood cells and the patient has severe anemia. Check with your doctor right ...
Anemias caused by decreased red blood cell (RBC) production have been grouped under the broad category of bone marrow failure, although their mechanisms and/or presentations may be markedly different. They include ():Pure red cell aplasia, either con
WASHINGTON -- The FDA updated the prescribing information of the anti-rejection drug mycophenolic acid (myfortic) to reflect a danger of pure red cell aplasia (PCRA).
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Transient erythroblastopenia of childhood (TEC) is a temporary red cell aplasia that occurs in young children, in the same time frame as primary infections. The cause is considered unknown, although viral infections are suspected, and parvovirus B19, enterovirus and HHV-6 have been documented in previous case reports.. A previous report of HHV-6 in TEC came two decades ago from the University of Pittsburgh. Investigators examined the bone marrow of 14 young children with hematologic disorders and found HHV-6 in the children with TEC but not in the children who did not have TEC (Penchansky 1997).. The investigators from Nagano Childrens Hospital in Japan are the first to show distinct erythroblast deformation by HHV-6 in TEC.. Read the full paper here.. ...
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Last night I had two intervals of sharp cramping pain. My lower back tightened up and was accompanied by a cold sweat and me wanting to vomit. I went upstairs after these two awful cramps to go potty and when I wiped, it was like someone had cut me down there. It was pure red blood... Nothing heavy but there was a blood clot and it was enough to scare me half to death and caused a crying jig on the toilet... I was begging God to keep the baby safe and growing strong. This was the first time I had bleed at all, so yes it freaked me out!! Through the night the blood turned to light pink then by this morning it was brown. I called the IVF clinic anyways and they said as long as it is not a heavy period (soaking pad kind) not to worry about it. They want me to call tomorrow to see how I am doing. I told them how sore I was waking up this morning. My uterus and insides felt like it has had a good workout! With my tummy sore and watching it change shape and position, it kind of throws you off a bit. ...
MM5T is a cellular matrix that is all about volume. This is the thing that makes Red Cell so special. Everything works because L-Arginine is allowed to take over, which enables your muscle cells to behave properly. Red-Cell Creating a Time Released Flood of Nitric Oxide.
This study will test whether the immune-suppressing drug rituximab can increase blood counts and reduce the need for transfusions in patients with moderate aplastic anemia, pure red cell aplasia, or Diamond Blackfan anemia. These are rare and serious blood disorders in which the immune system turns against bone marrow cells, causing the bone marrow to stop producing red blood cells in patients with pure red cell aplasia and Diamond Blackfan anemia, and red blood cells, white blood cells and platelets in patients with aplastic anemia. Rituximab is a laboratory-made monoclonal antibody that recognizes and destroys white blood cells called lymphocytes that are responsible for destroying bone marrow cells in these diseases. The drug is currently approved by the Food and Drug Administration for treating patients with B-cell non-Hodgkin lymphoma, a disease of white blood cells.. Participants receive four doses of rituximab, once a week for 4 weeks through a needle in an arm vein. The infusion rate ...
TY - JOUR. T1 - Recovery of erythropoiesis following allogeneic bone marrow transplantation for chronic lymphocytic leukaemia-associated pure red cell aplasia. AU - De Vetten, M. P.. AU - Van Gelder, M.. AU - De Greef, G. E.. PY - 2001. Y1 - 2001. N2 - Pure red cell aplasia is a rare condition, that can be either idiopathic or associated with a lymphoproliferative disorder. The latter is considered to result from T cell-mediated suppression of haematopoiesis, and usually responds well to treatment with immunosuppressive medication. We describe a patient with B-CLL-associated pure red cell aplasia who did not respond to several courses of immunosuppressive treatment. Erythropoiesis was finally restored after allogeneic bone marrow transplantation.. AB - Pure red cell aplasia is a rare condition, that can be either idiopathic or associated with a lymphoproliferative disorder. The latter is considered to result from T cell-mediated suppression of haematopoiesis, and usually responds well to ...
Anticytokine autoantibodies are an emerging mechanism of disease pathogenesis that have been shown to elicit a broad range of clinical phenotypes. Some anticytokine autoantibodies can lead to immune susceptibility, with examples including nontuberculous mycobacteria, salmonellae, or fungi due to autoantibodies against gamma interferon (IFN-γ) (1); staphylococcal infection due to anti-interleukin-6 (IL-6) autoantibodies (2); Burkholderia gladioli infection due to anti-IL-12p70 autoantibodies (3); chronic mucocutaneous candidiasis due to anti-IL-17 and anti-IL-22 autoantibodies (4, 5); and cryptococcal meningitis (6) or Nocardia infection due to anti-granulocyte-macrophage colony-stimulating factor (GM-CSF) autoantibodies (7). Other diseases in which immunodeficiency is not the primary presentation include the severe lung disease pulmonary alveolar proteinosis (PAP) caused by anti-GM-CSF autoantibodies; pure red cell aplasia (8); pure red-cell aplasia due to antierythropoietin autoantibodies (9); and
BACKGROUND: T-cell large granular lymphocyte (T-LGL) leukemia is a disorder only rarely reported in children. We diagnosed a new case of a clonal T-LGL proliferation in a 6-year-old girl presenting with severe neutropenia and pure red blood cell aplasia (PRCA). PROCEDURE: Flow cytometric analysis including TCR-Vbeta repertoire analysis and molecular studies using reverse transcriptase polymerase chain reaction (RT-PCR), PCR heteroduplex analysis and GeneScan analysis were performed to investigate the clonal nature of the T-LGL. RESULTS: Flow cytometric analysis revealed a Vbeta3 clonal nature. Molecular studies identified a clonal Vbeta3-Cbeta RT-PCR product. Both PCR heteroduplex analysis and GeneScan analysis found clonal TCRB and TCRG gene rearrangements. CONCLUSIONS: An underlying T-LGL leukemia should be investigated in the diagnostic evaluation of acquired PRCA and neutropenia in young children. Both flow cytometric and molecular analyses can be used to establish the clonal nature of T-LGL ...
Allogeneic hematopoietic stem cell transplantation (aHSCT) can cure patients with a variety of bone ... 2) Severe aplastic anemia (SAA) or pure red cell aplasia (PRCA [acquired or congenital]) associated with transfusion .... Clinical Trial last updated 04/28/2016 - 2:40pm.. ...
[Biological medicines of protein or polypeptide origin produced with biotechnology are far more complex in structure than the low molecular weight chemical ones. In conjunction with chemical drugs generic copies are completely the same, while in the field of biological medicines only similarity can be stated, as identical molecules cannot be produced. Spatial structure, isomers and side chains cause difference and for this reason these are called biosimilar drugs. Immunogenity of biosimilar drugs is very different and the risk of antibody production against them is diverse. Pure red cell aplasia, a rare side effect of erythropoietins is a life-threatening condition so every effort must be done for its prevention. Biosimilar drugs are not to be replaced with each other, and even the reference drugs should not be substituted in order to identify easily the side effects of each drug. Importantly financing should support these clinical principles namely a cheaper drug could be started as a new treatment but
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John MAA, Rhemtula YA, Menezes CN, GROBUSCH MP.Lamivudine-induced red cell aplasia. Journal of Medical Microbiology 57: 1032-1035, No. 8, Aug 2008 - South AfricaGoogle Scholar ...
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Guthrie, C. G. und Huck, J. G., On the existence of more than four isoagglutinin groups in human blood. Bull. Johns Hopkins Hosp. 1923. 34, 37-48; 34, 80 bis 88; 34, 128-135 (zit. nach 20).Google Scholar ...
TY - JOUR. T1 - Acute renal failure in a pediatric kidney allograft recipient treated with intravenous immunoglobulin for parvovirus B19 induced pure red cell aplasia. AU - Subtirelu, Mihail M.. AU - Flynn, Joseph T.. AU - Schechner, Richard S.. AU - Pullman, James M.. AU - Feuerstein, Dianne. AU - Rio, Marcela Del. PY - 2005/12/1. Y1 - 2005/12/1. N2 - Infection with parvovirus B19 (PV-B 19) after solid organ transplantation may cause pure red cell aplasia (PRCA). Intravenous immunoglobulin (IVIg) may be of benefit in clearing the infection. Acute renal failure is a known adverse effect of IVIg administration. A 14-yr-old male received a cadaveric renal transplant. Three weeks after surgery he developed symptomatic anemia (hemoglobin 4.5 g/dL, reticulocyte count 0.2%). Anti-PV-B19 IgM and IgG titers, which had been negative pretransplant, were positive. He received two IVIg infusions as treatment for the PV-B19 infection. Four days after the IVIg infusions he developed non-oliguric acute renal ...
Use CellCept exactly as prescribed by your doctor. Mycophenolate mofetil is reported to have a pKa values of 5. Study April 26, Baseball Great Rod Carew Owes His Life to NFL Players Transplanted Organs April 17, Before taking mycophenolate mofetil , tell your doctor or pharmacist if you are allergic to it; or to mycophenolic acid; or to mycophenolate sodium; or if you have any other allergies. Cases of pure red cell aplasia PRCA have been reported in patients treated with CellCept in combination with other immunosuppressive agents. Do not take any new medicine without talking with your doctor. CellCept Intravenous infusion solution must be prepared in two steps: The genotoxic potential of mycophenolate hearts game was determined in five assays. The highest dose tested was 0. CellCept has been administered in combination with the following agents in clinical trials: No data are kostenlos zocken online on the slotmaschinen gratis ohne anmeldung spielen of long-term exposure to this level of MPAG. ...
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Hypo-proliferative Anemias I. Problem/Condition. Hypo-proliferative anemia is an anemia where the bone marrows response, the production of reticulocytes, is absolutely low, or low for the degree of anemia (Reticulocytopenia). It is possible to have a hemolytic anemia or blood loss (a hyper-proliferative anemia) simultaneously, in which case the hemoglobin level will fall more rapidly. II.…. ...
True or False. Anemia, which can be as low as 5gm/dl, with reticulocytopenia can be associated with a serial rise in mean corpuscular volume over several months or years, and mild neutropenia and thrombocytopenia can be seen. ...
The HOXB13 gene has been implicated in prostate cancer (PrCa) susceptibility. We performed a high resolution fine-mapping analysis to comprehensively evaluate the association between common genetic variation across the HOXB genetic locus at 17q21 and PrCa risk. This involved genotyping 700 SNPs using a custom Illumina iSelect array (iCOGS) followed by imputation of 3195 SNPs in 20,440 PrCa cases and 21,469 controls in The PRACTICAL consortium. We identified a cluster of highly correlated common variants situated within or closely upstream of HOXB13 that were significantly associated with PrCa risk, described by rs117576373 (OR 1.30, P = 2.62×10−14). Additional genotyping, conditional regression and haplotype analyses indicated that the newly identified common variants tag a rare, partially correlated coding variant in the HOXB13 gene (G84E, rs138213197), which has been identified recently as a moderate penetrance PrCa susceptibility allele. The potential for GWAS associations detected through ...
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When blood flows slowly in a narrow tube, red-cell aggregation results in formation of an approximately cylindrical core of red cells, which moves as a rigid body. The core is denser than the surrounding fluid, and sedimentation is observed in horizontal tubes. To model this, the Stokes flow of a fluid surrounding a long solid cylinder (the core) contained in a long hollow cylinder (the tube) is considered. The cylinder axes are parallel but not coincident. An exact analytic expression for the resistance coefficient for motion perpendicular to the axes is given. This coefficient increases rapidly with the ratio of core radius to tube radius, and core eccentricity. The predicted rate of sedimentation is comparable to that observed experimentally. The apparent viscosity of a two-phase medium consisting of a core of aggregated particles and surrounding pure fluid is calculated. For a core radius corresponding to experimental conditions, the apparent viscosity increases rapidly with increasing ...
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BACKGROUND. Chimeric antigen receptor (CAR) T cells can induce remission in highly refractory leukemia and lymphoma subjects, yet the parameters for achieving sustained relapse-free survival are not fully delineated. METHODS. We analyzed 43 pediatric and young adult subjects participating in a phase I trial of defined composition CD19 CAR T cells (ClinicalTrials.gov, NCT02028455). CAR T cell phenotype, function, and expansion, as well as starting material T cell repertoire, were analyzed in relationship to therapeutic outcome (defined as achieving complete remission within 63 days) and duration of leukemia-free survival and B cell aplasia. RESULTS. These analyses reveal that initial therapeutic failures (n = 5) were associated with attenuated CAR T cell expansion and/or rapid attrition of functional CAR effector cells following adoptive transfer. The CAR T products were similar in phenotype and function when compared with products resulting in sustained remissions. However, the initial apheresed ...