ALBERT

Description: Introduction Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of NHL ~30% of newly diagnosed lymphoma cases. The International Prognostic Index (IPI) has been the primary clinical tool used to predict outcome for patients with large B-cell lymphomas (LBCL) and used in most clinical trials so far. This model identified five factors to predict DLBCL survival: age 〉60, elevated serum lactate dehydrogenase (LDH), ECOG performance status ≥2, Ann Arbor stage III or IV, and number of involved extranodal sites ≥2. This is a single center study aimed to study several clinicopathological characteristics of LBCL including: IPI parameters, double expressor status (DE) and patient's response at end of treatment by Positron emission tomography with 2-deoxy-2-[fluorine-18 ]fluoro- D-glucose integrated with computed tomography (18F-FDG PET/CT) (PET/CT scan). All relevant parameters were correlated to overall survival (OS) and event free survival (EFS). We found an excellent correlation between achievement of post treatment complete metabolic remission (CMR) by PET/CT and both OS and EFS while no statistically significant difference detected regarding OS or PFS among different IPI risk groups or in patients with DE phenotype compared to non-DE. Patient and Method: This is a 5- year mixed design study of adults with LBCLs. Baseline demographic, relevant laboratory data including LDH, pathological characteristics: cell origin of lymphoma, KI67 index, relevant immunohistochemical profile, DE status, achievement of CMR at end of treatment were correlated with OS and EFS at 2-years of diagnosis. Double expressor lymphomas (DELs) are defined by concomitant expression of MYC and BCL2 detected by IHC (cutoffs-40% MYC and 50% BCL2). Genetic double hit lymphomas (by FISH) were excluded. All patients in our cohort were treated with RCHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone) plus Rituximab regimen and evaluated at end of treatment by PET/CT scan using the Deauville five-point scale with scores of 1-3 considered negative and scores 4-5 is positive. All residual lesions exhibiting equal or less intense uptake than the liver were reported as CMR. PET/CT was done six to eight weeks after completion of chemotherapy and 12 weeks after the completion of radiation therapy. Results: Our cohort was composed of 170 patients diagnosed as large B cell lymphoma, the EFS and OS curves demonstrate the excellent outcome in patients treated with R-CHOP. No statistically significant difference in OS or PFS among different IPI risk groups (1-2 versus 3-5) in our cohort. Disease stage and LDH level had affected EFS with statistically significant differences but not OS. Other individual IPI parameters showed no significant correlation OS or PFS. Although the difference in survival between the two groups (DE and non-DE) are not statistically significant, it was clinically significant with a difference in survival between the two groups of approximately 24 months. OS and EFS among patients with CMR found to be significantly longer compared to those who did not achieve CMR with a statically significant difference (p:〈 0.05). CMR was noted to be higher among patients with favorable IPI (0 to 2) compared to patients with IPI values ranged between 3 - 5, however this difference was statistically insignificant (84.9 % vs 75.5%; P=0.178). The percentage of post treatment CMR was significantly higher among patients presented without extranodal disease compared to patients with extranodal involvement (94.1 % vs 74.1%; P=0.004). CMR was also significantly higher among patients presented with normal LDH compared to patients with high LDH (91.4 % vs 73.6%; P=0.009). The percentage of CMR was slightly higher among DE patients compared to non-DE patients , however this difference was statistically insignificant (83.3 % vs 79.6%; P=0.660). Conclusion While IPI is the most widely used prognostic predictor in LBCLs, there are limited data regarding the predictive value of post treatment PET/CT scans. We found that achievement of CMR at end of treatment was a very strong predictor of OS and PFS in our cohort compared to standard international prognostic parameters. To characterize the most important individual IPI parameters affected post treatment CMR and indirectly predicting OS and PFS, we found that extranodal involvement and LDH level were the most significant factors affecting CMR achievement. Figure. Figure. Disclosures No relevant conflicts of interest to declare.

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: Extra Medullary Hematopoiesis refers to the presence of hemopoietic elements in locations other than the bone marrow medullary space,It may be seen in many conditions, including chronic hemolytic anemias like thalassemia Intermedia or major. The common sites of involvement are the liver, spleen and the lymph nodes. Less common sites include the central nervous system, adrenal gland, kidney, perirenal soft tissues, breast, peritoneum and gastrointestinal tract the paraspinal region is a relatively uncommon location for these hemopoietic deposits. There are a few case reports of EMH occurring in the spineMost cases have been reported in connection with thalassemia, although it has also been described in patients with sickle cell anemia, polycythemia vera ,myelodysplastic syndrome and myelofibrosis. To the best of our knowledge, there are very few documented case reports in the literature of patients with myelofibrosis who developed cord compressive symptoms due to EMH in the spine from 1958 till 2016 only 15 cases were reported, In reported cases male to female ratio 7:1 with age range between 30 and 75 year old Paraprasesis are the most common presenting symptoms Thoracic region is the most affected site The degree of anemia and bone marrow fibrosis does not correlate with the occurrence of EMH since it was reported in cases with early fibrosis or mild anemia. Correlation between molecular markers like JAK2 and CALR, MPL is not described No definite guidelines have as yet been formulated for the treatment of such patients with EMH. Excision and decompression, radiotherapy, hypertransfusion and hydroxyurea alone or in combination have been proposed as well as lack of consensus about what is complete remission is it clinical or clinical and radiological and what is partial response is it any reduction of the mass lesion or any degree of clinical improvement in conclusion there is necessity to establish consensus for treatment and definition of CR, PR or No response in EMH with MF. Disclosures No relevant conflicts of interest to declare.

Description: Hairy cell leukemia (HCL) is an uncommon but distinct form of mature B-cell neoplasm that originates from activated late B-cells. It represents only 2% of all adult lymphoid leukemia; patients are predominantly middle-aged to elderly males with a median age of 50 years and is characterized by pancytopenia, monocytopenia and usually associated with massive splenomegaly. HCL associated with BRAF mutation 100% of cases, it's associated with hematological and oncological malignancies such as melanoma and papillary thyroid cancer with positive BRAF in 40 % of cases. Although the association of both cancers (HCL & papillary thyroid cancer) with BRAF mutation is well established in the literature, up to our knowledge, this specific combination has not been previously reported in one patient. Here we report a case of 48-year old Lebanese male, who presented to with bilateral hip pain and found to have lytic bone lesions on both x-ray and MRI. HIS CBC were normal and abdominal US didn't show any splenomegaly. Work-up for myeloma were negative. Bone marrow examination and flow cytometry results confirmed the diagnosis of hairy cell leukemia. The patient treated with cladrabine. Patient responded but have continues fever, PUO included Piston tomography showed abnormal uptake in thyroid. Ultrasound and final needle aspiration diagnose him as case of papillary thyroid cancer. He was treated with total thyroidectomy and followed up with RAI 30 micori. We sent BRAF from both bone marrow biopsy and thyroid tissue which turn out positive in both. The mutation results in substitution of adenine for thymine at position 1799 in exon 15 of the BRAF that replaces Valine (V) by glutamate (E) at amino acid 600(BRAF V600E). Although the BRAF V600E mutation is frequently present in different neoplasms, such as melanoma, papillary thyroid cancer, non-small cell lung cancer, colorectal cancer and Langerhans cell histiocytosis (X), within the lymphoid neoplasms, the BRAFV600E mutation is found to be highly specific for HCL and testing for this mutation is particularly useful in differentiating classic HCL from other B- cell neoplasm with overlapping features, such as HCL variant Mutation in BRAF (particularly V600E) in HCL remarkably increase the BRAF kinase activity renders the protein constitutively active, phosphorylating then ERK as a monomers independent from upstream regulatory signals or in a RAS-independent manner leading to constitutive activation of RAF-MEK-ERK signaling pathway and enhanced survival of leukemic hairy cells, similar to what occurs in other BRAF-mutated tumors as papillary thyroid carcinomas Other BRAF mutations outside exon 15 were rarely reported as exon 11 F468C and D449E mutations. We emphasize on the link of BRAF mutation in HCL and papillary thyroid cancer. The biology has been established but never in real clinical case. We recommend having high clinical suspicion and sending BRAF mutation in those types of cancers and link it with other possible abnormal findings, as might detect more cases of similar association. Disclosures No relevant conflicts of interest to declare.

Description: Chronic myeloid leukaemia (CML) is myeloprofilative neoplasm classically described as triphasic disease (chronic, accelerated and blast) and despite the significant efforts done by different groups to optimize treatment and outcome, still there is unmet needs and unanswered questions. Ophthalmologic Manifestations, is one of therapeutic challenges, the best available therapy for patient presenting with Ophthalmologic Manifestations, still unclear. One of the Uncommon presentation of CML is Ophthalmologic Manifestations which can can be variable from incidental finding during eye examination to blurred vision and partial or total loss of vision.There is paucity in the literature and a little is known about it, How it present? is ophthalmic examination for each patient newly diagnosed with CML is amust? Is there is cut off for leukocytosis which can predict eye damage?. Reversibility of this complications important from patient perspective, as well as both hematologist and ophthalmologist. The ophthalmological manifestations itself could have wide range of presentation and finding upon fundoscopy and eye examination. The pathological complications on the eye could be reversible or irreversible. The irreversible could be urgent like retinal hemorrhage or non-urgent like cotton wool spots, infections or proptosis. . Patients with ophthalmic manifestation of CML have been reported to have lower than 5-year survival rates Leukemic infiltrates most often affect the choroid; however, these are difficult to detect clinically and may present with a serious or exudative retinal detachment. Additionally, pallor and swelling of the optic nerve may indicate optic nerve infiltration and require immediate referral. By reviewing the literature, we found only 10 cases were reported to have ophthalmic manifestations . In 80 % of the cases eye symptoms and signs were first presentation of CML. The remaining were relapse. We notice The WBC average from (150- 450,000). There is no cut off in the literature for Hyperviscosity , neither for ophthalmological finding to happen. We observe from the cases review, all of them have had WBC at least more than 150,000. There no consensus to treat those patients, 10 % treated with surgery alone and another 10 % with radiotherapy alone, both scenarios were beforeTKI era. The remaining percentage of around 80% treated with combination of eye surgery with cytoreduction and TKI. Those patient who treated have had improved or resolved outcome.. We recommend base line fundoscopy and eye examination by ophthalmology as base line for all patients with new diagnosis of CML and WBC more than 150 on presentations or in the presence of symptoms or signs of ophthalmic manifestation.We believe that early intervention can save the patient eye. however base on reviewing the previous cases and input from ophthalmolopgist we proposed the following criteria (table)when doing eye exam to differentiate between the reversible versus irreversible since that can make big difference (table 1) and guiding the hematologist whether to use cytoreduction or to go ahead and do Leukopharesis ,however further prospective studies are needed to this unmet needs address and to consolidate the previous findings Disclosures No relevant conflicts of interest to declare.

Description: Extramedullary Hematopoisis refers to the presence of hemopoietic elements in locations other than the bone marrow medullary space, it may be seen in many conditions, including chronic hemolytic anemias like thalassemia Intermedia or major. The common sites of involvement are the liver, spleen and the lymph nodes. Less common sites include the central nervous system, adrenal gland, kidney, perirenal soft tissues, breast, peritoneum and gastrointestinal tract the paraspinal region is a relatively uncommon location for these hemopoietic deposits. There are a few case reports of EMH occurring in the spineMost cases have been reported in connection with thalassemia, although it has also been described in patients with sickle cell anemia,myelodysplastic syndrome and myelofibrosis. To the best of our knowledge, there are very few documented case reports in the literature of patients with polycythemia vera and Extra medullary Hemayopoiesis.The reported cases are limited in this regard only 11 cases were reported from 1979 till 2015, Paraprasesis is the most common presenting symptom, Age between 30 to 75 year with Male to female 6:1,Thoracic region is most affected site Most cases treated by surgery ,radiotherapy or both Poorer outcome compared to MF cases Overall the mortality in EMH with polycythemia vera is higher than MF and thalassemia, some of them due to fatal location of cord compression (cervical) No definite guidelines have as yet been formulated for the treatment of such patients with EMH. Excision and decompression, radiotherapy, hypertransfusion and hydroxyurea alone or in combination have been proposed as well as lack of consensus about what is complete remission is it clinical or clinical and radiological and what is partial response is it any reduction of the mass lesion or any degree of clinical improvement in conclusion there is necessity to establish consus for treatment and definition of CR ,PR or No response in EMH with polycythemia vera. Disclosures No relevant conflicts of interest to declare.

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 Chronic myeloid leukemia (CML), is a clonal bone marrow stem cell disorder that accounts for 10% of all leukemias. Generally, CML is one of the best examples of effective targeted therapy. Today, the overall survival of patients with CML treated by using tyrosine kinase inhibitors (TKIs) is very close to that of the healthy population. However, little is known about the effect of certain measures such as fasting especially during Ramadan period. In Islam, Ramadan fasting represents one of the five pillars of creed and considered as a mandatory religious duty, although in some instances among which, patients are exempted from observing this duty. The day fasting hours in Ramadan varies according to the geographical location of countries from 11 to 22 hours. To the best of our knowledge, this is the first quantatative study evaluating the effects of Ramadan fasting on patients with CML on TKIs. Objectives The main objective of this study was to evaluate the effect of fasting on CML patients receiving TKIs by evaluating their clinical course, hematological parameters, and BCR-ABL levels. Patients & Methods A retrospective study was conducted by reviewing medical records for adult Muslim patients with CML in the state of Qatar, who fasted Ramadan month during the period of 2016, 2017 and 2018, therefore we evaluated: 1) Complete Blood Count before, during, and after Ramadan. 2) BCR /ABL level before, during, and after Ramadan. 3) Any loss of hematological response or clinical evidence of disease progression like increase in spleen size during Ramadan. Patients were retrospectively asked and confirmed that they're fasting during Ramadan period by a telephone call made by the study team. Results A total of 49 patients fulfilled the criteria of the study, with median age of 46 years (range from 22-86), of these 36 (73.5%) were males and 13 (26.5%) were females, with ratio of 2.8 : 1. 27 (55.1%) patients are middle eastern, while 16 (32.7%) patients came from the Indian subcontinent, and 6 (12.25) patients were black. Multiple TKIs were used, imatinib was the most common TKI; used in 25 patients (51%), nilotinib in 15 patients (30.6%), dasatinib in 8 patients (16.3%) and ponatinib used only in one patient. Repeated measure analysis of variance (ANOVA) statistical analysis showed that the mean White blood count, neutrophils and BCR-ABL was found to be reduced after Ramadan period compared to before and during Ramadan period, however their difference was statistically insignificant (P〉0.05). Mean platelet, hemoglobin, basophils and eosinophils values appeared to have similar trend before, during and after Ramadan periods. Unpaired t test revealed that the mean values of the most of the hematological parameters among male was found to be higher compared to females. Age was found to be negatively correlated (low to moderate correlation) with all hematological parameters and BCR-ABL levels (Pearson correlation (r) ranges from -0.10 to -0.46). Conclusion TKIs use during fasting in Ramadan period is poorly studied with scarce data. The use of TKIs during fasting in Ramadan period did not result in significant changes in hematological indices nor in BCR-ABL levels in our study. Patients who wish to fast during Ramadan period may be reassured in this regard, yet physicians can adopt the safe trial approach, where they allow the patients to fast, but with instructions as when to break fasting. On the other hand, further research is needed to reach a definite conclusion. Table 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.