ALBERT

Description: Introduction: Hepatic iron concentration (HIC) is used as a surrogate for total iron balance to guide chelation therapy in transfusion-dependent and independent patients. Unfortunately, liver biopsy is invasive and provides only indirect information regarding other organ systems. FerriScanprovides an accurate validated measurement of liver iron concentration (LIC) through a non-invasive, using patented R2-MRI imaging technology. Aim: To determine the iron status of 11 patients with non-transfusion dependent (NT) patients with sickle cell disease (SCD). Patients and methods: FerriScan (a quick, easy and painless, with an MRI scan time of only two minutes) is used to determine LIC in eleven adults with NT-SCD. Serum ferritin, iron concentrations and hepatic enzymes (ALT and AST) concentrations and total iron binding capacity (TIBC) were measured. Results: 11 adults with NT-SCD were studied. Three had serum ferritin 〉 500 umol/L , 2 out of the three (ferritin level 1138 and 531 ug/L) had high liver iron measured by ferriScan (〉 30 mmol/kg dry tissue). One patient had high liver iron content despite a concomitant serum ferritin concentration = 237 ug/L. On the other hand a patient had serum ferritin = 1117 ug/L while his liver iron was still (27 mmol/kg dry tissue) in the normal range. Serum ferritin concentrations were correlated significantly with liver iron content measured by ferriScan (r = 0.47, p = 0.05). (fig) Three patients had elevated liver enzymes (ALT and AST). Neither serum ferritin, nor LIC was correlated significantly with hepatic function. Discussion: In this study significant number of patients with ND-SCD had high LIC and high serum ferritin and hepatic enzymes (ALT and AST). Elevated levels of LIC and ferritin impose high risk for hepatic disease and cardiac toxicity in these patients. Evidence suggests that patients with high LIC have higher risk of liver fibrosis and cirrhosis as a result of iron overload. In addition, Liver iron concentration (LIC) over 15.0 mg Fe/g dry weight is associated with increased risk of cardiac diseases. Moreover, the liver is considered the early warning system against later endocrine complications, due to iron overload. For NT-SCD, with increased LIC, effective management of liver iron concentration is critical to ensure risk of morbidity due to iron overload is minimized Summary: This is the first study that document increased iron overload in NT-SCD patients. Therefore, we recommend measuring serum ferritin and LIC in NT-SCD patients. Those with increased LIC and/or ferritin should be chelated to prevent long term complications of iron overload.Table.Ageserum FeTIBCFerritinliver ironALTASTyrumol/Lumol/Lug/Lmmol/kgU/LU/L32.323.755.7361.731.024.236.114.217.78.4405.717.716.422.3 Disclosures Nashwan: HMC MRC: Research Funding. Moustafa:HMC MRC: Research Funding. Elomry:HMC MRC: Research Funding.

Description: Thalassemia major (TM) requires chronic blood transfusions ultimately cause iron overload and subsequently end-organ damage unless corrected. Iron chelation has been proven to decrease organ dysfunction and improve survival in transfusion-dependent β-thalassemia. However, taking iron chelation therapy every day has sometimes been a challenge in patients. Deferasirox is a once-daily, oral iron chelator that developed out of a need for a long-acting chelator. The approved mode of administration requires taking deferasirox on an empty stomach with water, apple juice, or orange juice to limit variation in bioavailability. This required administration schedule might not be palatable for patients. Additionally, approximately one-quarter of patients experience mild to moderate gastrointestinal symptoms, which may pose additional challenges. Jadenu is a new oral formulation of Exjade tablets for oral suspension. While Exjade is a dispersible tablet that must be mixed in liquid and taken on an empty stomach ,Jadnu can be taken in a single step, with or without a light meal, simplifying administration of treatment and allows greater convenience and may be associated with fewer gastrointestinal side effects versus the original formulation. This may significantly improve compliance. In addition, the new formulation may be associated increased bioavailability. Jadenu is 36% more bioavailable than the original formulation, Exjade®. Therefore, to convert from Exjade to Jadenu the dose of Jadenu should be about 30% lower, rounded to the nearest whole tablet. To date, the new formulation of deferasirox has only been evaluated in pharmacokinetic studies in healthy volunteers. No clinical data are available yet in patients taking this formulation. The objective of this study was to compare the effect of Jadenu substituting Exjade on serum Ferritin concentration, liver iron content and biochemical profile in (BTM) patients with iron overload. Patients and Methods: Twelve adult patients with BTM were studied. All patients were on regular packed cell transfusion therapy monthly to keep their Hb not less than 9 g/dl before transfusion. They were on Exjade therapy (30 mg/kg per day) for 5 years or more before changing them to Jadenu therapy (14-28mg/kg/day). We evaluated Serum ferritin and the liver iron (LIC) measured by the Ferriscan method. Investigations included measuring hepatic functions (alanine transferase (ALT), aspartate transferase (AST), alkaline phosphatase (ALP) and albumin) , creatinine and fasting blood glucose (FBG) every clinic visit (q 3 months). In addition thyroid function (free T4 (FT4), thyrotropin (TSH), 25 OH vitamin D and PTH levels were measured before and one year after starting Jadenu therapy. Patients were monitored for gastrointestinal and other reported side-effects related to the drugs. All patients were on vitamin D 800 U/day and folic acid 5 mg / day. Paired t student test was used to compare lab results before versus after Jadenu treatment. Linear regression equation was used to investigate possible relation between variables. Results A year after treating patients with Jadenu serum ALT decreased (non-significant) but there was no significant change in circulating concentrations of creatinine, albumin, ALP or FBG. (Table 1) Apart from some gastrointestinal complaints reported in 3 patients that did not require discontinuation of therapy, patients did not have any other side effects. There was a non-significant decrease in LIC and ferritin levels after 1 year of using Jadenu. Thyroid and parathyroid hormone did not change during Jadenu therapy. (Table 2) A positive significant correlation was found between serum ferritin level and LIC measured by ferriscan method. LIC and serum ferritin level were correlated significantly with ALT level ( r = 0.31 and 0.45 respectively, p 〈 0.05) . No significant correlation was detected between LIC and other biochemical or hormonal levels. This study showed that the use of Jadenu after Exjade was associated with non-significant decrease in liver iron and ALT. There was no change in FBG, creatinine albumin or thyroid function. No side effects required discontinuation of the medicine. Conclusion: Jadenu is more palatable and improve quality of life for patients with BTM, however it was associated with minimal decrease in LIC and ALT level suggesting marginal improvement of iron chelation probably due to easier administration. Disclosures No relevant conflicts of interest to declare.

Description: BACKGROUND: Thalassemia is a heterogeneous group of inherited disorders of hemoglobin synthesis. It is a common disease in Mediterranean, Southeast Asia, Indian subcontinent, and Middle East countries, including Qatar. PURPOSE: The aim of this study was to assess the quality of life (QOL) among patients aged 14 to 18 years with thalassemia major (TM) in Qatar and correlates their QOL with bio-demographic data of the patients compared to healthy controls. MATERIALS AND METHODS: This cross-sectional study measured the QOL in adolescents with thalassemia major who were attending ambulatory units in a tertiary hospital in Qatar. Forty children and adolescents with TM and 40 healthy participants were enrolled in the study. Forty-two (52.5%) participants were males and 38 (47.5%) females. Data were collected utilizing PedsQLTM 4.0 generic core scale and were analyzed using the appropriate statistical method. RESULTS: Children with TM had significantly lower and more variable overall quality of life score (69.1 ± 16.8) compared to healthy matched children (77 ± 12.8), (p

Description: Almost 16,000 iron exposures annually are reported in children less than six years of age in the United States. Deferoxamine is the iron-chelating agent of choice. Deferoxamine binds absorbed iron, and the iron-deferoxamine complex is excreted in the urine. Indications for treatment include shock, altered mental status, persistent GI symptoms, metabolic acidosis, pills visible on radiographs, serum iron level greater than 500 µg/dL, or estimated dose greater than 60 mg/kg of elemental iron. No clear end point of therapy is distinguished. Infusion of deferoxamine for 6-12 hours has been suggested for moderate toxicity. For severe toxicity, administer deferoxamine for 24 hours. Because these end points are arbitrary, observe the patient for the recurrence of toxicity 2-3 hours after the deferoxamine has been stopped. Complications of iron toxicity include the following: Infection with Yersinia enterocolitica, acute respiratory distress syndrome (ARDS) and fulminant hepatic failure, hepatic cirrhosis, pyloric or duodenal stenosis. Systemic toxicity is expected with an ingestion of 60 mg/kg. Ingestion of more than 250 mg/kg of elemental iron is potentially lethal. Although a low serum ferritin is an accurate measure of iron deficiency, there is no accurate serum or plasma marker for acute body-iron overload. Serum iron concentration and transferrin saturation do not quantitatively reflect body iron. Stores and should therefore not be used as surrogate markers of tissue iron overload. Liver iron concentration provides the best measure of total body iron stores and is a validated predictor of the risks a particular patient faces from the complications of iron toxicity. Several imaging noninvasive techniques are available for measuring liver iron concentration (LIC) . There are two validated MRI methods for quantitating the liver iron burden: the FerriScan and T2 methods. The noninvasive R2-MRI technique (FerriScan) is highly sensitive and specific for estimating LIC and is approved by the Food and Drug Administration for routine clinical use. However, it was not used to diagnose and monitor LIC in cases of acute iron intoxication. This 27 year old female nurse by profession self-referred to hematology clinic for evaluation of Iron overload after self injecting herself with 20 ampoules of IV iron Ferro sac (each ampoule containing 200 mg of iron, (4000 mg elemental iron, 60 mg/kg) . Her CBC on presentation showed Hb of 12.5 g/dl her baseline Hb 9 g/dl with serum iron of 28 (NR 9.0 - 30.4 umol/L) ,TIBC of 42 NR(45 - 80 umol/L ), ferritin 1001 (NR 24-336 mcg/l) Her clinical exam was unremarkable. Her MRI showed severe iron overload. 9 mg /g dray tissue (NR 0.17-1.8) Patient received chelation with deferasirox at dose of 30 mg /kg for 6 months when her ferriscan showed almost normal LIC of 2 mg /g dry tissue. This case report showed the value of ferriscan in diagnosing the degree of tissue iron overload and in monitoring chelation to a safe level of hepatic iron content. Disclosures No relevant conflicts of interest to declare.

Description: The most accurate method with which to evaluate altered glucose metabolism in patients with TM is still controversial. Even if the annual oral glucose tolerance test (OGTT) by the age of 10 years is the recommended method, a diagnosis of 'normal' glucose tolerance during OGTT does not exclude abnormal postprandial glucose levels at home . There is now evidence that the OGTT method, evaluating fasting and 2-h post load glucose, may miss episodes of hyperglycaemia . Furthermore, the credibility of Hb A1c has been questioned because the hemoglobin composition of patients' erythrocytes are considerably modified, due to regular and frequent transfusions. The results may be falsely increased or decreased depending on the proximity to transfusion, shortened erythrocyte lifespan and the assay used . It has been demonstrated recently that the continuous glucose monitoring system (CGMS) is a useful and valid tool in defining glucose metabolism in children and adults affected by TM with early glucose derangements . Indeed, the CGMS allows monitoring of glycaemic profiles throughout a period of 72 h for a total of 288 glycaemic registrations per day. It identifies glycaemic excursions and constitutes a valid device to understand the 24-h glycaemic trend and profiles. Rimondiet al. investigated the value of using CGMS in six TM patients with abnormal glucose homeostasis after an oral glucose tolerance test (OGTT) . Two-hour OGTT glucose values and CGMS fluctuations were classified as normal if 〈 7.8 mmol/l, impaired if 7.8 to 11.1 mmol/l, diabetic if 〉 11.1 mmol/l. The TM patients spent from 1 to 23% of the time with a blood glucose level from 7.8 to 11.1 mmol/l. we evaluate three patients with Beta Thalassemai major using CGMS Patient 1 A 15 year old male with TM presented with nocturia. His FBG was 5.6 mmol/L and OGTT showed a BG level at 2hrs of 8.5 mmol/l. His CGMS showed a diabetic range of BG after dinner and overnight. Based on this tracing, a basal insulin (Glargine) was prescribed at night. A satisfactory response was recorded by CGMS . Patient 2 A 14 year old girl with TM with no symptoms related to glycemic abnormalities. Her FBG was 4.9 mmol/L and an OGTT showed a BG level at 2 hrs of 6.9 mmol/L (IGT). CGMS tracing showed prolonged persistent hyperglycemia after lunch suggesting a need for prandial insulin to cover her carbohydrate load. Insulin aspart before lunch properly controlled her glycemia . Patient 3 A 13 year old boy with TM had a normal FBG (4.5 mmol/L) and OGTT (2h BG =7.6 mmo/l). Applying CGMS monitoring showed IGT state. Furthermore, the effect of packed red cell transfusion showed marked reduction of his BG before meals and overnight. This is the first case to document the rapid beneficial effect of blood transfusion on glycemia in patiens with Beta thalssemia Major Advances in TM care have led to improved survival and quality of life in patients with TM; however, many chronic endocrine diseases have emerged as a result of potential endocrine complications by routine screening and a high index of suspicion is imperative for patients with TM to receive timely treatment.Our results demonstrate that the CGMS is a useful method to detect the variability of glucose fluctuations and offers the opportunity for better assessment of glucose homeostasis in TM patients. Proper and early iron chelation or the use of intensive iron chelation in those with high iron load the new oral chelators have been shown to decrease or reversethese glycemic abnormalities.In addition, optimization of insulin therapy with the help of CGMS appears to be clinically useful and costeffective approach. Disclosures No relevant conflicts of interest to declare.

Description: Background; Transfusion-dependent β-thalassemia (TDT) is a severe form of congenital anemia. Regular blood transfusion and continual administration of iron-chelators are conventional therapeutic strategies in TDT. Despite progressive advancements in developing various iron scavenging drugs, iron overload is still considered a challenging conundrum in these patients. Multiple organ dysfunctions result from the toxic effect of excess iron deposition in parenchymal tissues that cause high morbidity and mortality among TDT patients. The greatest affected tissues include endocrine glands, liver, heart, and kidneys. Aim of the study: To investigate pulmonary function of patients with TDT in relation to their iron overload. Patients and Methods: Pulmonary function tests were done for 10 Patient with TDT (table 1) in a well-equipped lung function unit in a tertiary care hospital by experienced respiratory technicians. An ideal test session was taken up following ATS/ERS recommendations. A proper grading system including acceptability and repeatability criteria was applied. Measurement of DLCO is done with a technique of single breath carbon monoxide (CO) uptake in the lung and lung volume is measured with gas dilution and body plethysmography. Chest x ray (CXR) was obtained in all patients. Pulmonary function test was classified according to the following categories based on ATS/ERS criteria and American medical association criteria (AMA); Normal: FEV1/FVC more than 70% FEV1, FVC, TLC, RV and DLCO normal (at least 80% predicted)Obstructive: FEV1/FVC less than 70% FEV1 and FVC (less than 80% predicted). TLC and RV either normal or elevated (at least 120% predicted). DLCO normal (at least 80% predicted)Restrictive: FEV1/FVC ratio (more than 70%) (1) FEV1, FVC, and TLC decreased (no more than 80% predicted) and a decrease in DLCO (no more than 80% predicted) or (2) TLC and RV decreased (no more than 80% predicted) with normal DLCO, FEV1, FVC, and FEV1/FVC, suggestive of low lung volumes, or (3) reduced TLC and DLCO.Mixed obstructive and restrictive: FEV1/FVC ratio reduced, suggestive of obstructive disease. TLC and RV reduced, suggestive of restrictive disease. DLCO normalIsolated low DLCO: DLCO decreased with normal FEV1, FVC, FEV1/FVC, TLC, and RV (As per AMA DLCO less than 75% were considered abnormal) Results: In our 10 TDT patients 9 had normal CXR. One patient had minimal patchy ground glass opacity. Six patients (60%) had abnormally low DLCO indicating some impairment at the level of alveolar capillary membrane. None of them had obstructive pattern. However 2 patients had some air trapping. DLCO, DLCO/Hb, DLCO/VA and RV were correlated negatively with LIC (fig 1) . PFTs (DLCO, RV, and TLC) were correlated negatively with the LIC. In addition, FEV1 and FVC were correlated negatively with ferritin level (r = -0.7 and -0.77 respectively, p

Description: Avascular necrosis of the femoral head is not specific disease. It occurs as a complication or secondary to various causes. These conditions probably lead to impaired blood supply to the femoral head. The diagnosis of AVNFH is based on clinical findings and supported by specific radiological manifestations. AVNFH occurs as a complication traumatic and non-traumatic disorders. Most cases of AVNFH are non-traumatic and occur secondary to excessive corticosteroid use and alcohol abuse.Other causes include coagulopathies, hemoglobinopathies (sickle cell disease), chronic liver disease ,gout, idiopathic hyperlipidemia, metabolic bone disorders, pregnancy, radiation, chemotherapy, smoking, systemic lupus erythematosus and vasculitis syndromes. Intravascular coagulation appears to be the central event associated with nontraumatic AVNFH , Coagulation may occur secondary to extravascular compression (marrow fat enlargement), vessel wall injury (chemotherapy, radiation), or a thromboembolic event (fat emboli). Magnetic resonance imaging MRI considered the preferred method for diagnosis of occult AVN, since it is more sensitive than bone scan or plain films. Due to the high incidence of bilateral AVN, MRI may pick up AVN in opposite asymptomatic hip. MRI has 90-100% sensitivity for symptomatic disease AVNFH can be presenting Manifestation for patient with CML as illustrated in table 1 Table 1.Chronic Myeloid Leukemia Presenting with Avascular Necrosis of Femur HeadPatientAgeGendersiteNoteYearReference124MaleRt Femoral HeadLeukocytecount 96,800/mm3, Platelets count 684,000/mm3, and Hemoglobin 10.4 g/dL.2005Moon JY, et al. (7)215FemaleRt Femoral HeadLeucocyte count of 290 X 109/L,Plateletcount 250 x 109/L, Hemoglobin 10.8 g/dl2003Gupta D, et al.(8)317MaleRt Femoral HeadUnknown1984Gibson J, et al.(9)49FemaleLt Femoral HeadLeukocytecount 359,000/mm3, Platelets count 809,000/mm31988Salimi Z, et al.(10)517MaleRt Femoral HeadUnknown1996Leone J, et al.(11)612FemaleRt Femoral HeadUnknown2013Leone J, et al.(12)Or can be consequence of therapy with interferon as illustrated in table 2or TKI as illustrated in table 3 Table 2. PATIENTS TREATED WITH INTERFERON-a Summary of Patients with Chronic Myeloid Leukemia and Associated AVNFH Patient Patient (yrs) Gender Interval from CML dx to development of AVNFH Platelet and WBC counts at time of AVNFH dx IFNa dose Other Rx Comment 1 22 Male 18 Platelets, 61-140x109/L; WBC, 2.5-3.5 x 109/L 5MU/q.o.d. to 2 MU 2x/week HU pegylated IFN, steroids x 1 week, anegrelide 2 45 Female 54 Platelets, 120-210 x 109/L; WBC, 15 x 109/L Varied from 10 MU/day to 5 MU/day HU, busulfan, ara-C (3 mos) 3 46 Female 6 Platelets, 160-220 x 109/L; WBC, 8.4-18 x 109/L 10 MU/day with concurrent ATRA and ara-C HU 4 17 Male Presenting symptom and symptoms recurred 1 month after starting IFNa and ara-c Platelets, 895 x 109/L; WBC, 167 x 109/L Unknown HU HU cytoreductiona/w clinical and radiographic improvement of AVNFH 5 25 Female 4 yrs Platelets, 1200 x 109/L;WBC 49 x 109/L Unknown HU ANFH developed when CML entered accelerated phase after 4 yrs of IFNa therapy Table 3. PATIENTS TREATED WITH TYROSINE KINASE INHIBITORS (TKIs) Summary of Patients with Chronic Myeloid Leukemia and Associated AVNFH Patient Patient (yrs) Gender Interval from CML dx to development of AVN Platelet and WBC counts at time of AVN dx TKI dose Other Rx Comment 1 12 Male 8 yrs WBC 5600/mm3 Start dose 400mg/d; escalated to 600mg/d to achieve complete cytogenetic response Dx as CML (chronic phase in 2005; started on imatinib 400mg (340mg/m2) after 20 months dose escalated to 600mg/day (continued for 1 yr) Nataraj V et al 2 34 female 3 year WBC 6000/ mm3 Failed Imatinib 400mg Then shifted to Dasatinib 100 mg Developed AVNFH18 months after Dastinib in (CHR, CCR, MMR)yassin et al Conclusion From the above mentioned review of literature; 6 patients with CML presented with AVNFH as the initial presentation prior any therapy; five in the era of interferon and two in era with TKIs; one with Imatinib and the other with Dasatinib treatment. Two issues to be considered; either the condition is rare or there is under-reporting of this side effect. Observational studies with proper reporting are required to accurately measure the incidence of this complication which could significantly affect patients' safety and quality of life. Disclosures Al-Dewik: Qatar National Research Fund (QNRF): Other: sponsorship.

Description: The diagnosis of beta thalassemia intermedia (BTI) is mainly based on the severity of clinical phenotype. It is associated with a wide range of specific complications including extramedullary hematopoiesis, leg ulcers, gallstones, hypercoagulable state, pulmonary hypertension (PHT), endocrine disorders and osteoporosis. The commonest endocrine complication in beta thalassemia major (TM) patients is hypogonadism followed by hypothyroidism and diabetes but the data on endocrine disorders in BTI patients are scarce. The aim of this study is to determine the prevalence of endocrine complications in a large series of BTI patients. Methods: In this multi-countries cross-sectional study, all BTI patients registered at 12 thalassemic treatment centers in Iran (2 Centers), Italy (2 Centers), Greece, Turkey (2 centers), Oman, Qatar, Jordan, Cyprus and United Kingdom were enrolled during 2017. Non transfusion- dependent beta-thalassemia patients or those who received blood transfusion 3-4 times or less annually, were evaluated. Required information was collected from medical records using a designed questionnaire. Demographic data, clinical characteristics and laboratory data included age, sex, splenectomy, type of treatment (blood transfusion, iron chelation, hydroxyurea), hormonal assays, bone mineral density, calcium -phosphorous metabolism, serum ferritin, liver function tests, fetal and total hemoglobin levels,, platelet and nucleated red blood cell counts, were collected. Results: A total of 721 BTI patients were enrolled in the survey from 9 countries. The most prevalent disease-related complications were osteoporosis (21.6%) and hypogonadism (12.6%) followed by: central hypothyroidism (8.3%), non-insulin-dependent diabetes mellitus (7.8%), primary hypothyroidism (5.5%), insulin-dependent diabetes mellitus (4.2%), hypoparathyroidism (2.2%), growth hormone deficiency (1.1%), adrenal mass (1%) and thyroid cancer (0.5%). Conclusion: This study evaluated the largest cohort of BTI patients with endocrine disorders. Although BTI patients are non-transfusion dependent or only occasionally transfused, iron-overload due to increased intestinal iron absorption and enhanced bone marrow activity cause endocrine disorders and osteoporosis. This study demonstrate that although endocrine complications are less common in patients with BTI compared to data reported in literature in TM patients , a regular monitoring with timely diagnosis and proper management underscoring on osteoporosis and gonadal disorders are crucial to prevent endocrine complications in these patients. Disclosures No relevant conflicts of interest to declare.

Description: Introduction: Priapism is a persistent penile erection not associated with sexual stimulation lasting for more than 4 hours. It is seen in up to 1.9% of patients with CML in some centers. Priapism adversely affects the quality of life, sexual function, of the affected patients. Long-lasting priapism is associated with a drastic effect on male fertility. The underlying pathophysiology involves disturbed autoregulation of the penile circulation through nitrous oxide (NO) and phosphodiesterase enzyme dysregulation. Method Literature searched in google scholar, PubMed, and Scopus search engines with keywords priapism, chronic myeloid leukemia, chronic granulocytic leukemia, and chronic myelogenous leukemia (English literature between 1960 to 2020) Results One hundred cases of priapism were reported in patients with CML. Most patients were below the age of 40 with mean 27.4years. The youngest was 7 weeks old and the oldest was 60 years old. Most patients had priapism as the first presentation of CM, 3 developed priapism after starting the treatment, 2 after stopping treatment, one noncompliant with treatment,1 previously diagnosed, and 1 patient post-splenectomy. The majority of patients' priapism occurred during the chronic phase of the disease and only 5/100 had priapism during the blast and accelerated phase. They had a mean WBC = 321.29 × 109/ (n=92), and mean platelet count = 569,000 (n=69). The lowest WBC count associated with priapism was 37 x10^9/L, the highest was 782 x10^9/L. Treatment modalities included medications, aspiration and irrigation of the corpora cavernosa, radiation to the penis, leukoreduction, and surgical shunts. Medications were used in (N = 50), aspiration of the corpora cavernosa in (N = 45) patients, leukapheresis in (N = 14), radiotherapy in (N= 9), and shunt in (N = 32). Surgical shunt relieved priapism in (N = 16), another 5 had shunt with partial response and needed chemotherapy to control the priapism. 19 patients responded to aspiration and irrigation, and another 2 required chemotherapy to control priapism. Radiation was helpful in resolving the priapism in 3 patients and Leukapheresis was useful in 5 patients. Medications alone were used to terminate priapism in 10 patients, however, priapism persisted for a longer duration compared to other modalities. It has been known that priapism lasting 〉 24 hours poses a high risk of permanent erectile dysfunction (94% or more). In 76 of CML patients with priapism, the mean time from onset to the presentation was 78.28 hours (three times longer than the high-risk period). The erectile function was affected in 24/100, not affected in 15/100, and not addressed in 61/100. Conclusion: Although priapism is an uncommon complication of CML, its fast diagnosis and management is crucial to avoid permanent erectile dysfunction. Disclosures No relevant conflicts of interest to declare.

Description: INTRODUCTION Iron deficiency is the most prevalent nutritional deficiency worldwide. Iron deficiency anemia (IDA) is the most common type of anemia, its prevalence is 1 out of 5 of the population. IDA is a well-known cause of reactive thrombocytosis which is mostly asymptomatic. Only few observational studies and case reports have described thromboembolic events in the context of this reactive thrombocytosis in the absence of other hypercoagulable states OBJECTIVES To assess the frequency of thromboembolic events in Arab patients with reactive thrombocytosis secondary to iron deficiency anemia (IDA). METHODS We retrospectively reviewed thromboembolic events in iron-deficient patients with reactive thrombocytosis. Our study sample included female patients who received iron replacement for IDA between April 2018 and March 2020 at Hamad Medical Corporation, Doha, Qatar. We excluded pregnant, non-Arab patients and patients under 18 or over 65 years of age. Reactive thrombocytosis was defined as thrombocyte count of more than or equal to 450 x 109 /L in the presence of iron deficiency anemia and ferritin level less than 30µg/l. RESULTS Out of 1567 patients (mean age =50 +/- 8.66 years) with the diagnosis of IDA, 292 (18.63%) had thrombocytosis. They had a mean platelet count = 534 +/- 121 x 109 /L). None of them had any symptom or sign of thromboembolic events. Discussion Thrombocytosis can be categorized into primary causes such as primary bone marrow disorders and myeloproliferative neoplasms, and secondary causes including infection, inflammation or drug-induced. Iron deficiency anemia leads to reactive thrombocytosis in a mechanism that is yet to be fully understood. The clinical impact of increased platelet counts is not well recognized in literature, and it has not been studied in the Arab population. Although reactive thrombocytosis has been generally considered benign, few case reports described thromboembolic events in patients with IDA reactive thrombocytosis. Few cases in the literature described thromboembolic events in reactive thrombocytosis with IDA. H. Z. Batur Caglayan et al published a case report of a 41-year-old Turkish female patient who presented with transient ischemic attack (TIA) due to intraluminal carotid artery thrombus, which was attributed to IDA-associated thrombocytosis. Another case series by P T Akins et al described three women with severe IDA and thrombocytosis secondary to menorrhagia who developed carotid artery thrombi. The mechanism by which low iron can affect thrombocyte count is still unknown. One study in mice and humans demonstrated that iron deficiency caused reduced megakaryocyte proliferation but increased ploidy independent of thrombopoietin. However, another study failed to identify the exact mechanism by which iron deficiency leads to increased platelet count. CONCLUSION Our study did not find any thromboembolic incident in a large number of patients with reactive thrombocytosis secondary to IDA diagnosed over two years in our Arab population. Disclosures No relevant conflicts of interest to declare.