Evaluation of The Therapeutic Regimen in COVID-19 in Transplant Patients: Where Do Immunomodulatory and Antivirals Stand?

Abstract Background The management of COVID-19 in organ transplant recipients is among the most imperative, yet less discussed, issues based on their immunocompromised status along with their vast post-transplant medication regimens. No conclusive study has been published to evaluate proper anti-viral and immunomodulator medications effect in treating COVID-19 patients to this date. Method: This retrospective study was conducted in Shiraz Transplant Hospital, Iran from March 2020 to May 2021 and included COVID-19 diagnosed patients based on SARS-CoV-2 RT-PCR positive test who had been hospitalized for at least 48 hours before enrolling in the study. Clinical and demographic information of patients, along with their treatment course and the medication used were evaluated and analyzed using multiple regression analysis. Results A total of 245 patients with a mean age of 49.59 years were included with a mortality rate of 8.16%. The administration of Remdesivir as an anti-viral drug (P-value˂0.001) and Tocilizumab as an immunomodulator drug (P-value < 0.001) could reduce the hospitalization period in the hospital and the intensive care unit, as well as the mortality rates significantly. Meanwhile, the patients treated with Lopinavir/Ritonavir experienced a lower chance of survival (OR < 1, P-value = 0.04). No significant difference was observed between various therapeutic regimens in clinical complications such as bacterial coinfections, cardiovascular and gastrointestinal adverse reactions, and liver or kidney dysfunctions. Conclusion The administration of Remdesivir as an anti-viral and Tocilizumab as an immunomodulatory drug in SOT recipients could be promising treatments of choice to manage COVID-19.

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Evaluation of the therapeutic regimen in COVID-19 in transplant patients: where do immunomodulatory and antivirals stand?

Virology Journal ◽

10.1186/s12985-021-01700-2 ◽

2021 ◽

Vol 18 (1) ◽

Author(s):

Mojtaba Shafiekhani ◽

Farbod Shahabinezhad ◽

Tahmoores Niknam ◽

Seyed Ahmad Tara ◽

Elham Haem ◽

...

Keyword(s):

Organ Transplant ◽

Solid Organ ◽

Transplant Recipients ◽

Organ Transplant Recipients ◽

Transplant Patients ◽

Clinical Complications ◽

Hospitalization Period ◽

Significant Difference ◽

Immunocompromised Status ◽

Immunomodulatory Drug

Abstract Background The management of COVID-19 in organ transplant recipients is among the most imperative, yet less discussed, issues based on their immunocompromised status along with their vast post-transplant medication regimens. No conclusive study has been published to evaluate proper anti-viral and immunomodulator medications effect in treating COVID-19 patients to this date. Method This retrospective study was conducted in Shiraz Transplant Hospital, Iran from March 2020 to May 2021 and included COVID-19 diagnosed patients based on SARS-CoV-2 RT-PCR positive test who had been hospitalized for at least 48 h before enrolling in the study. Clinical and demographic information of patients, along with their treatment course and the medication used were evaluated and analyzed using multiple regression analysis. Results A total of 245 patients with a mean age of 49.59 years were included with a mortality rate of 8.16%. The administration of Remdesivir as an anti-viral drug (P value < 0.001) and Tocilizumab as an immunomodulator drug (P value < 0.001) could reduce the hospitalization period in the hospital and the intensive care unit, as well as the mortality rates significantly. Meanwhile, the patients treated with Lopinavir/Ritonavir experienced a lower chance of survival (OR < 1, P value = 0.04). No significant difference was observed between various therapeutic regimens in clinical complications such as bacterial coinfections, cardiovascular and gastrointestinal adverse reactions, and liver or kidney dysfunctions. Conclusion The administration of Remdesivir as an anti-viral and Tocilizumab as an immunomodulatory drug in solid-organ transplant recipients could be promising treatments of choice to manage COVID-19.

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Prior Amiodarone Exposure Reduces Tacrolimus Dosing Requirements in Heart Transplant Recipients

Progress in Transplantation ◽

10.1177/1526924819835840 ◽

2019 ◽

Vol 29 (2) ◽

pp. 129-134 ◽

Cited By ~ 3

Author(s):

Nadine T. Breslin ◽

David M. Salerno ◽

Veli K. Topkara ◽

Farhana Latif ◽

Susan Restaino ◽

...

Keyword(s):

Heart Transplant ◽

Organ Transplant ◽

Patient Characteristics ◽

Tacrolimus Concentration ◽

Transplant Recipients ◽

Primary Graft Dysfunction ◽

Graft Dysfunction ◽

Transplant Patients ◽

Significant Difference ◽

And Control

Introduction: Amiodarone use prior to heart transplant is independently associated with a higher rate of severe primary graft dysfunction and in-hospital mortality. Amiodarone may also alter the pharmacokinetics of medications metabolized via cytochrome P450. No data exist regarding the interaction between pretransplant amiodarone and tacrolimus concentrations. Design: Single-center retrospective study of transplant patients between January 1, 2014, and June 30, 2016. A therapeutic tacrolimus concentration was defined as a trough level between 8 and 15 ng/mL for 2 consecutive days. The primary outcome was the tacrolimus therapeutic weight-based dosing requirements (mg/kg/day) for patients receiving amiodarone prior to transplant when compared to those without prior receipt of amiodarone. Secondary outcomes include the incidence of cellular rejection and mortality within 6 months posttransplant. Results: Multi-organ transplant recipients (n = 3), retransplants (n = 9), those who died prior to a therapeutic level (n = 1), and those receiving amiodarone posttransplant (n = 7) were excluded from the analysis. Of the 80 patients included, 34 (42%) received amiodarone prior to transplant. Patient characteristics were similar, with the exception of primary graft dysfunction incidence (38% in amiodarone vs 8.5% in control, P = .001). The median therapeutic dose was 0.1 (interquartile range [IQR]: 0.07-0.12) versus 0.13 (IQR: 0.09-0.17) in the amiodarone and control groups, respectively, ( P < .01). No significant difference in mortality or rejection was noted. Conclusion: Patients receiving amiodarone prior to transplant require a lower weight-based dose of tacrolimus.

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High-Dose Acyclovir for Cytomegalovirus Prophylaxis in Seropositive Abdominal Transplant Recipients

Annals of Pharmacotherapy ◽

10.1177/1060028017728296 ◽

2017 ◽

Vol 52 (1) ◽

pp. 5-10 ◽

Cited By ~ 5

Author(s):

Margaret R. Jorgenson ◽

Jillian L. Descourouez ◽

Glen E. Leverson ◽

Erin K. McCreary ◽

Michael R. Lucey ◽

...

Keyword(s):

Organ Transplant ◽

Invasive Disease ◽

High Dose ◽

Solid Organ ◽

Transplant Recipients ◽

Composite Failure ◽

Significant Difference ◽

Low Incidence ◽

Ganciclovir Resistance ◽

Long Term Impact

Background: Following abdominal solid organ transplant (aSOT), valganciclovir (VGC) is recommended for cytomegalovirus (CMV) prophylaxis. This agent is associated with efficacy concerns, toxicity, and emergence of ganciclovir resistance. Objective: To evaluate the incidence of high-dose acyclovir (HD-A) prophylaxis failure in seropositive aSOT recipients (R+). Methods: This was a retrospective, single-center study of R+ transplanted without lymphocyte-depleting induction between January 1, 2000, and June 30, 2013, discharged with 3 months of HD-A prophylaxis (800 mg 4 times daily). The primary outcome was incidence of prophylaxis failure. Secondary outcomes were incidence of biopsy-proven tissue-invasive disease and prophylaxis failure for each allograft subgroup. Results: A total of 1525 patients met inclusion criteria: 944 renal (RTX), 108 simultaneous pancreas-kidneys (SPK), 462 liver (LTX), and 11 pancreas (PTX) transplant recipients. The composite rate of HD-A prophylaxis failure was 7%; incidence of tissue-invasive disease was 0.4%. Failure rates were 4.5%, 6.1%, 11%, and 20% in the RTX, SPK, LTX, and PTX populations, respectively; tissue-invasive disease rates were 0.2%, 0%, 0.7%, and 10%. Failure occurred more frequently in the LTX and PTX populations ( P < 0.0001, HR = 2.6; P = 0.04 HR = 4.4). Incidence of tissue-invasive disease was minimal and not different in the RTX, LTX and SPK populations ( P = 0.34). When evaluating recipients of seronegative allografts (D−), the composite failure rate was 3.4% with no significant difference between allograft subgroups ( P = 0.45). Conclusion: HD-A may be a reasonable prophylaxis alternative for D−/R+ recipients, in the absence of lymphocyte-depleting induction, if low incidence viremia is tolerable. Future studies are needed to determine the long-term impact of CMV viremia in the setting of this prophylaxis approach.

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Neurocognitive Effects of Solid Organ Transplantation

Cognitive and Behavioral Abnormalities of Pediatric Diseases ◽

10.1093/oso/9780195342680.003.0053 ◽

2010 ◽

Author(s):

Nataliya Zelikovsky ◽

Debra S. Lefkowitz

Keyword(s):

Organ Transplantation ◽

Survival Rates ◽

Organ Transplant ◽

Solid Organ Transplant ◽

Chronic Illnesses ◽

Solid Organ ◽

Transplant Recipients ◽

Transplant Patients ◽

Neurocognitive Outcomes ◽

Medical Advances

The first successful organ transplant was a kidney transplant performed between identical twins in 1954. Since that time, major medical advances have been made to help improve survival rates for transplant recipients. In 2008, there were 1,964 solid organ transplants performed for children under age 18 (2007 Organ Procurement and Transplantation Network and the Scientific Registry of Transplant Recipients [OPTN/SRTR] Annual Report 1997–2006). Currently, approximately 1,830 pediatric patients are awaiting some type of solid organ transplant (2007 OPTN/SRTR Annual Report 1997–2006). Organ transplantation in children is relatively recent compared to other treatments for children with chronic illnesses. The focus over the first few decades has been on medical advances and improving survival rates for transplant patients. In the recent years, increasing attention has been given to the developmental, neurocognitive, and psychosocial outcomes prior to transplant and in the short-term period post transplant. Most chronic illnesses and acute traumatic medical events have implications for neurocognitive outcomes. End-stage disease of the liver, kidney, heart, and lung are all believed to affect intellectual, academic, and neurocognitive functions. Gross neurodevelopmental deficits have become less common due to early medical intervention (e.g., improved nutrition, surgical intervention, reduced exposure to aluminum (Warady 2002). Organ transplantation is believed to ameliorate the deleterious long-term developmental and neurocognitive effects, but this topic has received little attention in the literature, and the available results with regard to intellectual, academic, and neurodevelopmental results have been mixed. In a combined sample of solid organ transplant patients, 40% had clinically significant cognitive delays (Brosig et al. 2006). Examining the impact of different underlying disease processes and transplantation of each solid organ separately is critical. Thus, we discuss the neurocognitive outcomes of each organ group separately in this chapter. Neurocognitive outcomes can be assessed in a variety of ways depending upon the age of the child. Among infants and toddlers, neurocognitive functioning is measured by an assessment of motor function, social and environmental interaction, and language development. Assessment of older children may involve the evaluation of intelligence, academic achievement, emotional and behavioral functioning, and adaptive skills.

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P1763TUBERCULOSIS IN RENAL TRANSPLANT RECEPIENTS - DO RITUXIMAB AND OTHER IMMUNOSUPRESSION HAVE A ROLE?

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p1763 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Anupma Kaul ◽

Dharmendra Bhaduria ◽

Narayan Prasad ◽

Amit Gupta

Keyword(s):

Renal Transplantation ◽

Renal Transplant ◽

Immunosuppressive Agents ◽

Induction Agent ◽

Renal Transplant Recipients ◽

Transplant Recipients ◽

Transplant Patients ◽

Significant Difference ◽

High Prevalence ◽

Mean Time

Abstract Background and Aims Rituximab is an anti CD 20 agent used widely in renal transplant recipients. Its use is associated with various infections;however, its association with Tuberculosis (TB) is not well established and has not been studied in post renal transplantation patients. Method This is a single centre, retrospective analysis of 56 renal transplant recipients who received rituximab for various reasons and 287 post renal transplant patients who did not receive rituximab during the study period from January 2013 to June 2017. The association between use of rituximab and incidence of TB was studied. Other factors associated with tuberculosis were also investigated. Results Baseline characteristics were similar in both the groups. Mean time for occurrence of TB was 18.4 + 10.6 months after renal transplantation. Rituximab use was not significantly associated with tuberculosis or any other infection. Higher number of rejection episodes (60% vs 32.72%, p=0.029) was the only factor associated with greater incidence of TB. However, no specific type of rejection was associated with tuberculosis. Use of plasmapheresis in post transplant period for treatment of humoral rejections was associated with significantly higher incidence of TB (33.33% vs 13.41%, p=0.031), however when pre- transplant plasmapheresis was also considered, there was no significant difference. The choice of induction agent was not associated with higher incidence of TB. Conclusion Use of rituximab is not associated with higher incidence of TB when compared to other immunosuppressive agents. Routine screening and prophylaxis may not be advisable especially in a country like India with high prevalence of TB; as it will further delay transplantation and may adversely affect the outcome of the patients.

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Discordance of SHEA/IDSA Clostridium difficile Disease Severity Scale in Solid Organ Transplant Patients

Open Forum Infectious Diseases ◽

10.1093/ofid/ofx163.949 ◽

2017 ◽

Vol 4 (suppl_1) ◽

pp. S383-S383 ◽

Cited By ~ 1

Author(s):

Tiffany Lee ◽

Christopher McCoy ◽

Carolyn D Alonso ◽

Graham M Snyder ◽

Christin Rogers ◽

...

Keyword(s):

Clostridium Difficile ◽

Organ Transplant ◽

Solid Organ Transplant ◽

Solid Organ ◽

Research Support ◽

Transplant Patients ◽

Cell Counts ◽

Immunocompromised Status ◽

White Blood Cell Counts ◽

Healthcare Epidemiology

Abstract Background Solid organ transplant (SOT) patients are at high risk for Clostridium difficile infections (CDI) due to chronic immunosuppression and a propensity to receive antimicrobials. Management of CDI in SOT patients poses unique challenges as this population has disease-altered clinical and laboratory parameters. The objective of this study was to assess concordance between various CDI severity scales and the Society for Healthcare Epidemiology of America/Infectious Diseases Society of America (SHEA/IDSA) guidelines. Methods This retrospective study included all SOT recipients with a first CDI episode following transplant and time-matched (2:1) to non-SOT patients experiencing first CDI episodes between 2008 and 2016. The primary endpoint was concordance rates of CDI episodes considered mild-moderate or severe/severe-complicated in published CDI scales compared with the SHEA/IDSA guidelines. We also sought to compare the distribution of CDI severity across all scales between SOT and non-SOT patients. Results Overall, 32 SOT patients and 64 non-SOT patients were included. The SOT group had significantly higher leukopenia rates at CDI diagnosis; however, the magnitude of serum creatinine change did not differ between groups. According to the SHEA/IDSA scale, CDI episodes in SOT recipients were categorized as mild-moderate and severe/severe-complicated in 23 (72%) and 9 (28%) patients, respectively. Overall concordance rates among SHEA/IDSA guidelines and other scales ranged from 28% to 72%. Concordance rates were highest for mild-moderate CDI with Belmares and for severe/severe-complicated CDI with ESCMID (Table 1). No scale evenly categorized SOT and non-SOT patients across all severities (Figure 1). Conclusion Severity scales with heavy emphasis on white blood cell counts may not adequately categorize SOT patients. Immunocompromised status may need to be considered on its own when categorizing CDI severity and prescribing therapy. Disclosures C. D. Alonso, Merck: Grant Investigator and Scientific Advisor, Research grant sanofi pasteur: Investigator and Scientific Advisor, Research support GSK: Investigator, Research support; E. B. Hirsch, Merck: Grant Investigator, Grant recipient The Medicines Company: Speaker’s Bureau, Speaker honorarium

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Transplant-Associated Skin Cancer: Role of Reducing Immunosuppression

Journal of the National Comprehensive Cancer Network ◽

10.6004/jnccn.2007.0047 ◽

2007 ◽

Vol 5 (5) ◽

pp. 541-549 ◽

Cited By ~ 10

Author(s):

Marcy Neuburg

Keyword(s):

Skin Cancer ◽

Therapeutic Strategy ◽

Organ Transplant ◽

Solid Organ ◽

Transplant Recipients ◽

Cutaneous Malignancy ◽

Transplant Patients ◽

Historic Review ◽

Solid Organ Transplant Recipients

This article explores the role of reducing immunosuppression as a therapeutic strategy for the problem of transplant-associated skin cancer. The specific issue of immunosuppression reduction is based on a brief historic review of the epidemiology of skin cancer in transplant patients, followed by a description of the role of immunosuppression as a cause of skin cancer. Finally, the literature pertaining to the hypothesis that reducing immunosuppression in solid organ transplant recipients favorably impacts both the incidence of cutaneous malignancy and outcomes relating to individual aggressive malignancies is presented.

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P1794CHANGES IN PHARMACOKINETIC PROFILE OF MYCOPHENOLATE MOFETIL AND TACROLIMUS IN THE TRANSPLANTED PATIENT AFTER BOWEL SURGERY: A PROSPECTIVE COHORT STUDY

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p1794 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Nuria Montero ◽

Alexandre Favà ◽

Anna Manonelles ◽

José González Costello ◽

Edoardo Melilli ◽

...

Keyword(s):

Organ Transplant ◽

Intestinal Resection ◽

Pharmacokinetic Profile ◽

Cardiac Transplant ◽

Solid Organ ◽

Transplant Recipients ◽

Post Dose ◽

Transplant Patients ◽

Bowel Surgery ◽

Terminal Ileostomy

Abstract Background and Aims Considering the particular pharmacokinetic (PK) profile of mycophenolate (MMF/MPS) with the important contribution of enterohepatic recirculation (EHC) and the potential alteration in tacrolimus (TAC) exposure, a PK study in solid-organ transplant patients who had undergone intestinal resection was carried out. Method This is a prospective single-center study of MMF/MPS and TAC exposure changes after bowel resection and after reconstruction. Whole blood samples were collected at the following time points: 0, 0.5, 1, 1.5, 2, 4, 6, 8 and 12 hours post-dose. Areas under the curves (AUCs) were determined in both conditions: with ileostomy and after bowel reconstruction. Results Six renal and two cardiac transplant recipients were included. Four subjects completed both pre- and post-reconstruction surgery procedures. Different intestinal anatomic resections were performed (Table 1). Patients with terminal ileostomy showed an under exposure to MMF/MPS. In three patients, initial MPA levels were on target, but they decreased >80% after 4 hours post-drug administration. After bowel reconstruction, the AUC increased maintaining MMF/MPA levels during 12h (Table 2). Before bowel reconstruction, TAC trough levels were within therapeutic target but, after reconstruction, AUCs normalized by dose were much higher than the expected. Conclusion Transplant recipients with ileostomy showed infra-exposure to mycophenolate. After 4 hours post-dose, MMF/MPA was undetectable because of the absence of EHC, which was recovered after anatomical correction. TAC exposure was higher after bowel reconstruction suggesting changes in the absorption. The use of mTORi in such clinical situations would be an alternative.

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Incidence of Heparin Induced Thrombocytopenia in the Transplant Population: A Large Retrospective Cohort, Single Transplant Centre Experience

Blood ◽

10.1182/blood.v120.21.3320.3320 ◽

2012 ◽

Vol 120 (21) ◽

pp. 3320-3320

Author(s):

Syed Hassan ◽

Dania Khoulani ◽

Ami Badami ◽

Zaid Alirhayim ◽

Mohamad A. Younes ◽

...

Keyword(s):

Retrospective Cohort ◽

Conflicts Of Interest ◽

Organ Transplant ◽

Serotonin Release ◽

Confirmatory Test ◽

Thrombin Inhibitor ◽

Thromboembolic Events ◽

Transplant Recipients ◽

Heparin Induced Thrombocytopenia ◽

Transplant Patients

Abstract Abstract 3320 Objective: Heparin induced thrombocytopenia (HIT), a prothrombotic complication of heparin therapy, can lead to serious thromboembolic events and cause significant morbidity and mortality. Its occurrence has never been studies in transplant patients, where use of heparin products is very common. We aim to study its prevalence in the transplant population at our institute. Methods: This is a retrospective cohort, single center study which looked into the clinical and laboratory database of all the patients that has undergone any kind of transplant at our institution over a period of 25 years (January 1985 - December 2010). In patients with clinical suspicious of HIT, a pre-test probability was calculated using the 4T scoring system. Results of the laboratory test like the ELISA HIT antibody (HIT ab) test and the functional serotonin release assay test (SRA) along with clinical manifestation of skin necrosis or thromboembolic events were reviewed. Results: Medical records of 2800 patients that has undergone transplant from January 1985- December 2010 were reviewed. HIT antibody assay was performed in 262 patients in which HIT was suspected. Of these, only 48 (18%) patients (mean age 57 ± 11 years, 71% women) had HIT ab positive, 9 were pre transplant recipient and remaining 39 were post transplant recipients. Baseline characteristics of the transplant population are illustrated in Table.1. Confirmatory test, SRA was performed in 8 HIT antibody positive patients, of whom only 4 were positive. The mean 4T score in HIT suspected patients was 3.7 ±1.3, while the score in HIT ab positive patients was 4.2 ± 1.2. Thrombotic complications were seen in 11(0.4%) patients, with the highest incidence rate of 1% in heart transplant recipients. No transplant patient had skin manifestations. Direct Thrombin inhibitor (DTI) was used only in 5 patients who had thrombotic events. No other complication or mortality was reported in any of the HIT ab positive transplant patients. Conclusion: To our knowledge, this is the first study of its kind that has shown very low incidence of HIT in transplant population. In conclusion, transplant patients can safely undergo any type of organ transplant, without having any peri or post operative complications or immediate mortality related to HIT. Disclosures: No relevant conflicts of interest to declare.

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Clinical Complications in Adult Patients with Sickle Cell Anemia and β-Thalassemia-Sickle Cell Disease

Blood ◽

10.1182/blood.v120.21.4767.4767 ◽

2012 ◽

Vol 120 (21) ◽

pp. 4767-4767

Author(s):

Giovanna Graziadei ◽

Alessia Marcon ◽

Martina Soldarini ◽

Ilaria Gandolfi ◽

Luisa Ronzoni ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Sickle Cell Anemia ◽

Acute Chest Syndrome ◽

P Value ◽

Cell Disease ◽

Transfusion Therapy ◽

Clinical Complications ◽

Significant Difference ◽

The Mean

Abstract Abstract 4767 Background. Sickle-Cell Disease (SCD) is one of the most common severe monogenic inherited disorders worldwide, due to hemoglobin S (HbS), with reduced affinity for the oxygen. HbS polymerization, leading to erythrocyte rigidity, vaso-occlusion and hemolytic anemia, is central in the pathophysiology and crucial for the clinical outcome. The term SCD refers to Sickle Cell Anemia (SCA) due to homozygosis for βS allele, HbS/β-thalassemia (T-SCD) due to compound of β-thal and βS allele, and HbSC disease, owing to the coinheritance of βS and βcalleles. SCD is a multiorgan disease characterized by recurrent acute events and progressive organ damage, worsening during the life. Aims. This is a retrospective monocentric study aimed to assess and compare the clinical complications among 59 adult SCD patients, followed at the Hereditary Anemia Centre of the Foundation IRCCS “Ca Granda” Ospedale Maggiore Policlinico, in Milan, Italy. Methods. Mutation analysis of the b globin gene was established by direct DNA sequencing on the ABI Prism 310 genetic analyzer. Clinical and hematological features were evaluated by routine tests and physical examination, with special attention to the erythropoiesis stress parameters as LDH values and extramedullary erythropoietic (EE) masses. Results. Fifty-nine adult SCD patients, 16 SCA and 43 T-SCD, were evaluated. In T-SCD patients detected b-mutations were severe (b°) in 69.8%, and moderate or mild (b+-b++) in 30.2%. The mean age of SCA patients was 36±9 and 41±11 years for T-SCD patients. For both groups the mean follow-up was 20±6 years, while the mean age at the presentation in our Centre was 32±8 years in SCA patients and 31±10 years in T-SCD ones. Five out of 16 (31.2%) SCA patients and 16/43 (37.2%) T-SCD patients were male. HbF mean levels were 6.9±5.1% and 10.1±7.2%, respectively in SCA and T-SCD group; surprisingly Hb mean levels were lower in SCA (9.3±1.3 g/dl) than in T-SCD (9.9±1.4 g/dl) patients. Comparing SCA and T-SCD, there was statistically significant difference in splenic features: splenectomy was performed in 2/16 (12.5%) SCA patients vs 21/43 (48.8%) T-SCD patients (p-value < 0.01). Splenomegaly was absent in SCA, while was detected in 11/22 (50%) T-SCD (p-value < 0.0001); all SCA patients had functional asplenia, not observed in T-SCD patients; splenic infarctions were absent in SCA patients and were detected in 7/22 (31.8%) T-SCD patients, of whom 5 had splenomegaly and 2 had normal spleen size (pvalue <0.001). On the other side, there was not statistically significant difference in the prevalence of stroke, acute chest syndrome (ACS), bone pain crisis, sepsis, leg ulcers and priapism. However, we observed some clinical differences, even if not statistically significant. Cholecistectomy was performed in 4/16 (25%) SCA patients vs 17/43 (39.5%) T-SCD patients, and gallstones were detected respectively in 5/12 (41.7%) and in 14/26 (53.8%) of SCA and T-SCD patients. Thrombotic events were absent in SCA patients, compared to 4/43 (9.3%) T-SCD patients. Furthermore, we detected EE in 3/16 (18.6%) SCA and in 3/43 (7%) T-SCD, all carrying b° thal mutations. We underlie that Hb levels and LDH values were higher in SCA than in T-SCD patients (823±295 vs 689±209 U/L). About the treatment, 14/16 (87.5%) SCA and 31/43 (72%) T-SCD underwent to top-up transfusion; 5/43 (11.6%) T-SCD were regularly transfused. Seven out of 16 (43.8%) SCA and 18/43 (41.8%) T-SCD patients were treated with Hydroxycarbamide (HU). Criteria for transfusion therapy were: painful crisis not responsive to HU, major clinical complications, such as stroke or ACS, extramedullary erythropoietic masses associated with high LDH levels and low Hb values. Conclusions. These data suggest that SCA and T-SCD patients have similar clinical course. Splenomegaly is present only in T-SCD patients, probably due to the increased amount of extravascular hemolysis. Surprisingly, SCA patients showed EE and lower Hb levels with higher LDH values compared to T-SCD ones. This could be related to the prevalence of intravascular hemolysis, that can lead to erythropoietic stress in SCA, even if tissues are better oxygenated in these patients because of biochemical characteristic of HbS in terms of decreased oxygen affinity. These observations could be important to evaluate transfusion and HU treatment. Disclosures: Cappellini: Novartis: Research Funding.

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