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Multiple myeloma (MM) is an incurable disease, whose clinical heterogeneity makes its management challenging, highlighting the need for biological features to guide improved therapies. Deregulation of specific long non-coding RNAs (lncRNAs) has been shown in MM, nevertheless, the complete lncRNA transcriptome has not yet been elucidated. In this work, we identified 40,511 novel lncRNAs in MM samples. lncRNAs accounted for 82% of the MM transcriptome and were more heterogeneously expressed than coding genes. A total of 10,351 overexpressed and 9,535 downregulated lncRNAs were identified in MM patients when compared with normal bone-marrow plasma cells. Transcriptional dynamics study of lncRNAs in the context of normal B-cell maturation revealed 989 lncRNAs with exclusive expression in MM, among which 89 showed de novo epigenomic activation. Knockdown studies on one of these lncRNAs, SMILO (specific myeloma intergenic long non-coding RNA), resulted in reduced proliferation and induction of apoptosis of MM cells, and activation of the interferon pathway. We also showed that the expression of lncRNAs, together with clinical and genetic risk alterations, stratified MM patients into several progression-free survival and overall survival groups. In summary, our global analysis of the lncRNAs transcriptome reveals the presence of specific lncRNAs associated with the biological and clinical behavior of the disease.

Nivolumab is a monoclonal antibody directed against programmed cell death-1 receptor. It has an increasing application in the treatment of various advanced metastatic cancers. The incidence of autoimmune side effects associated with such agents is expected to increase. New-onset autoimmune diabetes mellitus associated with immune checkpoint inhibitor treatment is rare, occurring in less than 1% of patients. Nivolumab-induced diabetes often presents as diabetic ketoacidosis, which could be life-threatening if not recognized and treated promptly. We present the case of a patient who developed severe diabetic ketoacidosis concomitant with hyperosmolar hyperglycaemic state (HHS) after receiving nivolumab for metastatic testicular lymphoma. Pre-nivolumab blood glucose levels were normal, apart from transient hyperglycaemia related to steroids as part of the chemotherapy protocol. The diagnosis was confirmed with extremely low C-peptide in the clinic. LEARNING POINTS: Checkpoint inhibitor-associated diabetes can present abruptly with life-threatening complications. Most patients require multiple daily injections of insulin upon discharge. Cessation of checkpoint inhibitor therapy does not revert diabetes.

Background Plasma cell neoplasms can show aberrant expression of different lineage-related antigens; however, co-expression of T-cell-associated markers on malignant plasma cells is extremely rare. Material and methods This report describes clinicopathologic characteristics of three myeloma patients with emergent plasmablastic morphology and aberrant acquisition of T-cell-associated markers diagnosed in our center. An extensive literature search for similar cases was conducted, and the relevant pathologic, clinical, and prognostic characteristics were summarized. Results A total of 22 cases of plasma cell neoplasm (including the three cases reported here) showed aberrant co-expression of T-cell markers. We found an evident association between aberrant expression of T-cell markers on malignant plasma cells and extramedullary involvement, aggressive morphologic features, high proliferative index ki67 >90%, aggressive clinical course, an adverse outcome, and short survival. Discussion & Conclusion Due to the rarity of this aberrant phenotype and scarcity of the published data, the precise causative mechanism and its clinical implications have not yet been elucidated.

This is a report of a very rare case of marginal zone lymphoma in a 15-years old male with Ataxia Telangiectasia. Besides being a rare diagnosis within the pediatric age group, this case exhibited a true challenge not only from diagnostic point of view but also regarding management plans. Marginal zone lymphoma is an indolent disease in which transformation is extremely rare particularly the pediatric subtype. However, there isn't enough data regarding marginal zone lymphoma in a sitting of immunodeficiency conditions had been published so far. The case described here showed some features pointing to more aggressive disease with massive bone marrow involvement and areas of focal transformation unlike the subtle pattern of infiltration that is usually seen in cases of marginal zone lymphoma. Another interesting finding in our case is the presence of a clone of CD4/CD8 double positive T-cells of moderate size (12%) with restricted expansion of Vb1 region. The significance of this clone is uncertain as it could be a transient reactive clone or a pre-malignant one. Keywords: Ataxia telangiectasia, Marginal zone lymphoma, Low grade lymphoma

Background Neurolymphomatosis is rare. Neoplastic lymphocytes are seen to invade nerves (cranial or peripheral), nerve roots or other related structures in patients with hematological malignancy. It is a separate entity from central nervous system lymphoma. Neurolymphomatosis has most commonly been described in association with B-cell non-Hodgkin lymphoma. Neurolymphomatosis in the context of Burkitt lymphoma and the post-renal transplant setting has not been described before. Case reports We report for the first time in the Arabian Gulf countries and nearby Arab states four cases of neurolymphomatosis (one Asian, and the other 3 are from Arabic nationals) occurring between 2012 and 2017 involving the median nerve, optic nerve, nerve root and cauda equina in patients with Burkitt lymphoma, Philadelphia chromosome-positive B-cell acute lymphoblastic leukemia and diffuse large B-cell lymphoma. Conclusions Neurolymphomatosis is rare and can be difficult to diagnose by biopsy but reliably confirmed by a combined imaging approach. Prior treatment with high-dose dexamethasone might suppress 18F-fluorodeoxyglucose (FDG) activity and decrease the sensitivity of positron emission tomography/computed tomography (PET/CT). The prognosis is generally poor but using high-dose methotrexate as well as high-dose chemotherapy and autologous stem cell transplantation may be an effective way to treat neurolymphomatosis.

Around the world, cancer care services are facing many operational challenges. Operations management research can provide important solutions to these challenges, from screening and diagnosis to treatment. In recent years, the growth in the number of papers published on cancer care operations management (CCOM) indicates that development has been fast. Within this context, the objective of this research was to understand the evolution of CCOM through a comprehensive study and an up-to-date bibliometric analysis of the literature. To achieve this aim, the Web of Science Core Collection database was used as the source of bibliographic records. The data-mining and quantitative tools in the software Biblioshiny were used to analyze CCOM articles published from 2010 to 2021. First, a historical analysis described CCOM research, the sources, and the subfields. Second, an analysis of keywords highlighted the significant developments in this field. Third, an analysis of research themes identified three main directions for future research in CCOM, which has 11 evolutionary paths. Finally, this paper discussed the gaps in CCOM research and the areas that require further investigation and development.

Introduction: In the era of routine use of positron emission tomography/computed tomography (PET/CT) for staging, it is not yet clear whether PET/CT can replace bone marrow biopsy for the assessment of bone marrow involvement in large B-cell lymphoma. Objectives: To compare the clinical utility of bone marrow biopsy and PET/CT scanning in the staging of large B-cell lymphoma. Methods: This was a retrospective analysis of all patients who presented to single center over a 4-year period with large B-cell lymphoma who had concurrent PET/CT and bone marrow biopsy performed in the assessment and staging of the lymphoma. Results: Out of 89 patients, 24 had bone marrow involvement either by PET/CT, by bone marrow biopsy, or by both. Bone marrow biopsy identified 12 patients (sensitivity 50%, specificity 100%, negative predictive value 84%), whereas PET/CT identified 23 patients (sensitivity 96%, specificity 100%, negative predictive value 98%). No patients were upstaged by the bone marrow biopsy result, and no patients had their treatment plan changed based on the bone marrow biopsy result. Conclusion: The results show that PET-CT is more sensitive and has better negative predictive value than bone marrow biopsy. This suggests that PET-CT could replace bone marrow biopsy in detecting bone marrow involvement for staging of large B-cell lymphoma.

The current study retrospectively evaluated cytogenetic profiles, various prognostic factors, and survival outcomes in 128 acute myeloid leukemia (AML) patients (14 ≤ age ≤ 70 years) admitted to the National Center for Cancer Care and Research (NCCCR), Hamad Medical Corporation, Doha, Qatar, between January 2010 and December 2016. The median age at diagnosis was 43 years, and 80% were less than 60 years old; 75% of patients were male. Cytogenetic analysis was integrated into the World Health Organization 2008 classification and showed that the percentages of normal and abnormal karyotypes were similar, accounting for 48.4% of each group of patients. The AML risk stratification based on cytogenetic analysis resulted in the following distribution: 18% in the favorable risk group, 57% in the intermediate-risk group, 24% in the unfavorable risk group, and 1% unknown. Only 88 patients received therapy with curative intent; 67% achieved complete remission, increasing to 81% after inductions 1 and 2. The median overall survival (OS) and disease-free survival (DFS) in AML patients were 26.6 and 19.5 months, respectively. The 3-year OS and DFS were 40 and 36%, respectively. Prognostic factors including age, gender, white blood cell count, and risk stratification were not significantly associated with treatment outcomes, whereas response to treatment vs. failure was significantly associated with the outcome (p = 0.01). The current study supports the importance of cytogenetics as a useful tool in diagnosis, prognosis, and risk assessment in AML treatment.

International audience; Scleromyxedema is a rare skin and systemic mucinosis that is usually associated with monoclonal gammopathy (MG). In this French, multicenter, retrospective study of 33 patients, we investigated the clinical and therapeutic features of MG-associated scleromyxedema. Skin molecular signatures were analyzed using a transcriptomic approach. Skin symptoms included papular eruptions (100%), sclerodermoid features (91%), and leonine facies (39%). MG involved an IgG isotype in all patients, with a predominant λ light chain (73%). Associated hematologic malignancies were diagnosed in 4/33 patients (12%) (smoldering myeloma, n=2; chronic lymphoid leukemia, n=1; and refractory cytopenia with multilineage dysplasia n=1). Carpal tunnel syndrome (33%), arthralgia (25%) and dermato-neuro syndrome (DNS) (18%) were the most common systemic complications. One patient with mucinous cardiopathy died of acute heart failure. Intravenous immunoglobulin (HDIVig) treatment alone or in combination with steroids appeared to be quite effective in nonsevere cases (clinical complete response achieved in 13/31 patients). Plasma cell-directed therapies using lenalidomide and/or bortezomib with dexamethasone and HDIVig led to a significant improvement in severe cases (HDIVig-refractory or cases with central nervous system or cardiac involvement). The emergency treatment of DNS with combined plasmapheresis, HDIVig, and high-dose corticosteroids induced the complete remission of neurological symptoms in 4/5 patients. Quantitative reverse transcriptase-PCR (RT-PCR) analysis of 6 scleromyxedema skin samples showed significantly higher profibrotic pathway levels (transforming growth factor β (TGFβ) and collagen-1) than in healthy skin. Prospective studies targeting plasma cell clones and/or fibrotic pathways are warranted for long-term scleromyxedema management.

Background Plasma cell neoplasms can show aberrant expression of different lineage-related antigens; however, co-expression of T-cell-associated markers on malignant plasma cells is extremely rare. Material and methods This report describes clinicopathologic characteristics of three myeloma patients with emergent plasmablastic morphology and aberrant acquisition of T-cell-associated markers diagnosed in our center. An extensive literature search for similar cases was conducted, and the relevant pathologic, clinical, and prognostic characteristics were summarized. Results A total of 22 cases of plasma cell neoplasm (including the three cases reported here) showed aberrant co-expression of T-cell markers. We found an evident association between aberrant expression of T-cell markers on malignant plasma cells and extramedullary involvement, aggressive morphologic features, high proliferative index ki67 >90%, aggressive clinical course, an adverse outcome, and short survival. Discussion & Conclusion Due to the rarity of this aberrant phenotype and scarcity of the published data, the precise causative mechanism and its clinical implications have not yet been elucidated.

Multiple myeloma (MM) is a plasma cell neoplasm associated with a broad variety of genetic lesions. In spite of this genetic heterogeneity, MMs share a characteristic malignant phenotype whose underlying molecular basis remains poorly characterized. In the present study, we examined plasma cells from MM using a multi-epigenomics approach and demonstrated that, when compared to normal B cells, malignant plasma cells showed an extensive activation of regulatory elements, in part affecting coregulated adjacent genes. Among target genes up-regulated by this process, we found members of the NOTCH, NF-kB, MTOR signaling, and TP53 signaling pathways. Other activated genes included sets involved in osteoblast differentiation and response to oxidative stress, all of which have been shown to be associated with the MM phenotype and clinical behavior. We functionally characterized MM-specific active distant enhancers controlling the expression of thioredoxin (TXN), a major regulator of cellular redox status and, in addition, identified PRDM5 as a novel essential gene for MM. Collectively, our data indicate that aberrant chromatin activation is a unifying feature underlying the malignant plasma cell phenotype. This research was funded by the European Union's Seventh Framework Programme through the Blueprint Consortium (grant agreement 282510), Fundació La Marató de TV3, Instituto de Salud Carlos III (ISCIII) PI14/01867, PI16/02024, and PI17/00701, TRASCAN (EPICA), MINECO Explora (RTHALMY), Departamento de Salud del Gobierno de Navarra 40/2016, Gilead Fellowship Program (GLD16/00142), Multiple Myeloma Research Foundation Networks of excellence, the International Myeloma Foundation (Brian van Novis) and the Qatar National Research Fund award 7-916-3-237. Furthermore, the authors acknowledge the support of the Generalitat de Catalunya Suport Grups de Recerca AGAUR 2017-SGR-736 and 2017-SGR-1142, TRASCAN-iMMunocelland European Research Council starting grant (MYELOMANEXT), CIBERONC (CB16/12/00489, CB16/12/00369 and CB16/12/00225), co-financed with FEDER funds, the Accelerator award CRUK/AIRC/AECC joint funder-partnership, as well as NCI R01 CA180475 and a MMRF collaborative grant. R.O. was supported by a FPU Fellowship of the Spanish Government, M.K. by an AOI grant of the Spanish Association Against Cancer and N.R. by the Acció instrumental d'incorporació de scientífics i tecnòlegs PERIS 2016 from the Generalitat de Catalunya. This work was partially developed at the Centro Esther Koplowitz (CEK, Barcelona, Spain). We particularly acknowledge the patients for their participation and the Biobank of the University of Navarra for its collaboration.

Sanguinarine (Sang), a plant-derived compound isolated from the roots of Sanguinaria canadensis was evaluated for its potential pro-apoptotic effects in precursor B acute lymphoblastic leukemia (Pre-ALL) cell lines. Treatment of 697, REH, RS4;11, and SupB15 cell lines with Sang exhibited significant inhibition of cell viability via induction of apoptotic cell death. Sang-mediated apoptosis was found to be associated with the increased expression of proapoptotic bax with concomitant decrease of Bcl-2 expression leading to depolarization of mitochondria membrane resulting in loss of mitochondrial membrane potential (MMP). The reduced MMP caused the leakage in mitochondrial membrane and release of cytochrome c into the cytosol. The cytochrome c then mediates the activation of caspase-cascade and subsequently PARP cleavage. Furthermore, pretreatment with z-VAD-FMK, a pan-caspase inhibitor, abrogated Sang-induced inhibition of cell viability, induction of apoptosis. Sang treatment also reduced the phosphorylation of AKT and suppressed the expression of a number of anti-apoptotic genes such as cIAP1, cIAP2, and XIAP. Sang mediates its anti-cancer activity by generation of reactive oxygen species (ROS) due to depletion of glutathione level in leukemic cell lines. Pretreatment of these cells with N-acetyl cysteine (NAC) prevented Sang-induced depletion of glutathione level and mitochondrial-caspase-induced apoptosis. Finally, Sang treatment of Pre-ALL cell suppressed colony formation ability of these cells suggesting Sang has an anti-leukemic potential. Altogether, our data suggest that Sang is an efficient inducer of intrinsic apoptotic cell death via generation of ROS and exhibition of anti-leukemic effect in Pre-ALL cells raises the possibility to develop Sang as a therapeutic modality for the treatment and management of Pre-ALL. This study was supported by Medical Research Center [RP # 16354/16], Hamad Medical Corporation, Doha, and State of Qatar. Scopus

No abstract, as A letter to the Editor.

Introduction: Scleromyxedema (rare cutaneous mucinosis), is characterized by the formation of lichenoid papules and presence of Serum monoclonal IgG in most cases, or all; after repeated testing. Patient concerns : The patient is a 51-year-old male presented with thick, disfiguring elephant-like erythematous skin folds over the forehead, papular shiny eruptions over ears and trunk and waxy erythematous papules over arms and hands without dysphagia or respiratory or neurologic symptoms Diagnosis : Skin biopsy from right arm was consistent with scleromyxedema. Serum cryoglobulin was reported negative. Complete blood count and routine blood biochemistry were normal. Thyroid function tests were normal. Serum protein electrophoresis and immunofixation showed monoclonal band of 14.5 g/L typed as IgG lambda. Interventions : Our patient was refractory to lenalidomide however improved clinically on immunoglobulins infusions on monthly basis without change in the MGUS level. Outcomes : NGF analysis revealed approximately 0.25% Lambda monotypic plasma cells in the bone marrow expressing CD38, CD138, and CD27 with aberrant expression of CD56 and were negative for CD45, CD19, CD117, and CD81. We also detected 0.002% circulating plasma cells (PCs) in peripheral blood. Conclusion : The immunophenotype of circulating tumor cells (CTCs) remain close to the malignant PCs phenotype in the BM. Hence, we report NGF approach as a novel diagnostic tool for highly sensitive MRD detection in plasma cell dyscrasias including scleromyxedema.

Sub population of cancer cells, referred to as Cancer stem cells (CSCs) or tumor initiating cells, have enhanced metastatic potential that drives tumor progression. CSCs have been found to hold intrinsic resistance to present chemotherapeutic strategies. This resistance is attributed to DNA reparability, slower cell cycle and high levels of detoxifying enzymes. Hence, CSCs pose an obstacle against chemotherapy. The increasing prevalence of drug resistant cancers necessitates further research and treatment development. The current review presents the essential mechanisms that impart chemoresistance in CSCs as well as the epigenetic modifications that can induce drug resistance and considers how such epigenetic factors may contribute to the development of cancer progenitor cells, which are not killed by conventional cancer therapies.

Tuberculosis (TB) can present with various forms and can occasionally be mistaken for malignancy. Hereby, we report a 53-year-old man diagnosed and treated for Burkitt's lymphoma in 2009 who achieved a complete remission confirmed by a computed tomography (CT) scan. During the follow-up 2 years later, he complained of left hip pain that warranted investigation with magnetic resonance imaging and whole-body 18F-fludeoxyglucose-positron emission tomography (FDG-PET)/CT which showed a benign lesion in the left hip associated with multiple lymph nodes in the chest and abdomen not amenable for biopsy. A follow-up PET/CT scan a few months later showed intense tracer uptake in the lymph nodes with size progression and appearance of new lymph nodes suspicious of lymphoma relapse. The patient was asymptomatic, and all investigations including viral and connective tissue disease studies were negative. Also the tuberculin skin test and QuantiFERON were negative. Lymph node biopsy was planned; however, the patient presented a few days earlier with fever, headache and photophobia. Cerebrospinal fluid (CSF) examination confirmed meningitis with lymphocytic pleocytosis and elevated protein. The CSF Gram stain, culture, viral and acid-fast bacilli were negative. CSF flow cytometry and cytopathology confirmed polyclonal lymphocytosis and suggested reactive causes. CSF TB culture grew Mycobacterium tuberculosis. Mediastinal lymph node biopsy also confirmed TB lymphadenitis. Four antituberculosis drugs were started. One year later, a PET/CT scan showed regression of all the involved lymph nodes. This case highlights the importance of excluding TB in patients with suspected malignancy, especially if they belong to endemic regions, and the increasing role of 18F-FDG-PET/CT in the early detection of extrapulmonary TB.

Objective Idiopathic thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder mediated by autoantibodies directed against ADAMTS13. This provides a rationale for the use of rituximab in this disorder. We report our experience and the outcome of 10 cases of TTP (9 refractory and 1 relapsing) successfully treated with rituximab in combination with plasma exchange (PE) and other immunosuppressive treatments. Methods The diagnosis of TTP was based on clinical criteria and supported by severe deficiency of ADAMTS13 activity and presence of inhibitors in seven cases. Rituximab was started after a median of 18.6 sessions of PE (range: 5-35) at the dose of 375 mg/m2/week for 4-8 weeks. Results Complete remission was achieved in all patients after a median time of 14.4 days of the first dose (range: 6-30). After a median follow-up of 30 months (range: 8-78), eight patients were still in remission and two developed multiple relapses, treated again with the same therapy, and achieved complete responses; they are alive, and in complete remission after a follow-up of 12 and 16 months. Conclusion Rituximab appears to be a safe and effective therapy for refractory and relapsing TTP. However, longer follow-up is recommended to assess relapse and detect possible long-term side effects of this therapy.

According to World Health Organization (WHO) classification (2008), B-cell neoplasms are classified into precursor B-cell or a mature B-cell phenotype and this classification was also kept in the latest WHO revision (2016). We are reporting a male patient in his fifties, with tonsillar swelling diagnosed as diffuse large B-cell lymphoma (DLBCL), germinal center. He received 6 cycles of RCHOP and showed complete metabolic response. Two months later, he presented with severe CNS symptoms. Flow cytometry on bone marrow (BM) showed infiltration by CD10-positive Kappa-restricted B-cells with loss of CD20 and CD19, and downregulation of CD79b. Moreover, the malignant population showed Tdt expression. BM Cytogenetics revealed t(8;14)(q24;q32) within a complex karyotype. Retrospectively, MYC and Tdt immunostains performed on original diagnostic tissue and came negative for Tdt and positive for MYC. It has been rarely reported that mature B-cell neoplasms present with features of immaturity; however the significance of Tdt acquisition during disease course was not addressed before. What is unique in this case is that the emerging disease has acquired an immaturity marker while retaining some features of the original mature clone. No definitive WHO category would adopt high-grade neoplasms that exhibit significant overlapping features between mature and immature phenotypes.

Hematopoietic stem cell transplantation (HSCT) offers potentially curative therapy for many hematologic and nonhematologic conditions. As a successful outcome of Qatar’s National Cancer Strategy, the HSCT program was started in the National Center for Cancer Care and Research (NCCCR) in October 2015. The HSCT program in NCCCR is the only transplant program in Qatar and self-sufficient with all three core components: the stem cell collection facility, the stem cell processing facility, and the clinical program, which are locally available at Hamad Medical Corporation. In this paper, we report on the outcomes of the first 16 patients who underwent autologous stem cell transplantations (ASCTs) in our center. A total of 17 ASCT have been performed for 16 adult (≥14 years) patients. Thirteen of the 16 patients were eligible for disease evaluation at Day 100 post-ASCT. Among these patients, the overall response rate on Day 100 was 92% (complete remission, 61%; very good partial remission/partial remission, 31%) and stable disease occurred in 6%. The procedure was very well tolerated by all patients. At the time of writing this report, all patients are alive; however, one patient (6%) had disease relapse. The Day 100 post-ASCT nonrelapse mortality rate was 0%. Launching the HSCT program represents a historic milestone in the development of the health-care sector in Qatar. The 1st year of this program was very fruitful with the accomplishment of 17 successful transplants. We are in the process of starting the allogenic HSCT early next year. This would represent the next significant milestone for cancer care in Qatar. Keywords: Autologous stem cell transplantations, Hematopoietic stem cell transplantation, Qatar

Multiple myeloma (MM) is a plasma cell neoplasm associated with a broad variety of genetic lesions. In spite of this genetic heterogeneity, MMs share a characteristic malignant phenotype whose underlying molecular basis remains poorly characterized. In the present study, we examined plasma cells from MM using a multi-epigenomics approach and demonstrated that when compared to normal B cells, malignant plasma cells showed an extensive activation of regulatory elements, in part affecting co-regulated adjacent genes. Among target genes upregulated by this process, we found members of the NOTCH, NFkB, mTOR1 signaling and p53 signaling pathways. Other activated genes included sets involved in osteoblast differentiation and response to oxidative stress, all of which have been shown to be associated with the MM phenotype and clinical behavior. We functionally characterized MM specific active distant enhancers controlling the expression of thioredoxin (TXN), a major regulator of cellular redox status, and in addition identified PRDM5 as a novel essential gene for MM. Collectively our data indicates that aberrant chromatin activation is a unifying feature underlying the malignant plasma cell phenotype.

Acquired pure megakaryocytic aplasia is a rare hematological disorder characterized by thrombocytopenia with absent or markedly reduced megakaryocytes in the bone marrow. We report a case of a 25-year-old male diagnosed as acquired pure megakaryocytic aplasia. Treatment with prednisone and intravenous immunoglobulin failed, but he was successfully treated with cyclosporine, with complete remission after 90 days and normal platelet count maintained thereafter.Kazanılmış saf megakaryositik aplazi, kemik iliğinde namevcut ya da anlamlı ölçüde azaltılmış megakaryosit içeren trombositopeni ile karakterize, nadir hematolojik bir hastalıktır. Kazanılmış saf megakaryositik aplazi teşhisi konmuş, prednizon ve intravenöz immunoglobulin ile tedavisi başarısız olmuş ve siklosporin ile başarılı biçimde tedavi edilerek 90 gün sonra tam remisyona ulaşan ve ondan sonra platelet sayımı normalleşen 25 yaşında erkek bir olgunun raporu sunulmuştur.

Strongyloides stercoralis (S.S.) is a human intestinal parasite, which may lead to complicated strongyloidiasis. We report a case of disseminated strongyloidiasis following the treatment of myeloma. The patient developed skin lesions, respiratory distress, aseptic meningitis and bacterial and fungal sepsis. The diagnosis of strongyloidiasis was established through endotracheal tube secretions. Despite the treatment with Ivermectin and Albendazole, the outcome was fatal. The value of screening for strongyloidiasis is unclear but may be of benefit in patients with hematological malignancies from high endemic areas. Keywords: acute respiratory failure, disseminated strongyloidiasis, myeloma, steroids

Viral retinitis due to cytomegalovirus (CMV) infection is rare in patients with acute leukemia who did not receive hematopoietic stem cell transplantation. We report a case of CMV retinitis that developed in a 49-year-old patient with acute lymphoblastic leukemia. The patient was treated with salvage chemotherapy using a hyper-CVAD regimen and did not receive hematopoietic stem cell transplantation. The incidence of CMV retinitis in this subgroup of patients is not described in literature. He had a very complicated course during chemotherapy but was successfully treated, with preservation of visual acuity, and to date he is in complete remission. Interestingly, prior to CMV retinitis, the patient had been diagnosed with and treated for candida retinitis. This case shows the importance of eye examination and care in patients diagnosed with hematological malignancies.

Objectives: To report the rates of cardiomyopathies in the population below 50 years of age in Qatar. Subjects and Methods: We conducted a retrospective review of clinical data on patients with cardiomyopathy who were hospitalized in Hamad General Hospital, Doha. Data were collected from medical records during the 1996–2002 period and prospectively from the patients who were hospitalized during the year 2003. All cardiomyopathy patients below 50 years of age who were citizens or permanent residents in Qatar were included in this study. Results: During the study period, a total of 132 cases were recorded with idiopathic cardiomyopathies. Among these, 67.4% were males and 32.6% females; Qatari 31.8%, non-Qatari 68.2%. The consanguinity rate was high among Qatari patients. In the first 7-year study period, 1996–2002, the incidence rate of all types of cardiomyopathies was 2.5/100,000 population per year (95% CI: 1.4–3.5). It increased to 5.2/100,000 population during the year 2003 (95% CI: 3.6–6.7). Dilated cardiomyopathy was most prevalent (75.8%) in all age groups, and the incidence increased remarkably with age. Lower prevalence of hypertrophic cardiomyopathy (13.6%) and left ventricle noncompaction cardiomyopathy (6.1%) was found. In children below 15 years of age, the incidence rate for all types of cardiomyopathies was 2.7/100,000 population. The overall mortality rate was 5.3%. Conclusion: Most cases of cardiomyopathy were identified at an early age: below 15 years and above 35 years of age. Introducing preventive and early diagnosis programs may have an impact on reducing the mortality and morbidity from idiopathic cardiomyopathy.

Mantle cell lymphoma is an aggressive B-cell neoplasm with special clinical and pathological characteristics. As the morphological variants of mantle cell lymphoma are currently acknowledged, a practical challenge for achieving the correct diagnosis is encountered especially when the cytogenetic/molecular data are not widely available. Here we describe a case of mantle cell lymphoma (MCL) presented with a blend of atypical clinical, morphological, and immunophenotypic features that led to an incorrect diagnosis of B-cell prolymphocytic leukemia (B-PLL).

Abstract 4384 AML is a group of neoplastic disorders resulting from clonal expansion of immature hematopoietic cells in the bone marrow, blood and other tissues. AML represents approximately 90% of all acute leukemias in adults. The median age at diagnosis is 65 years. Geographic variations in the incidence and subtype of AML have been reported in different parts of the world. The classification of AML has evolved from the standard morphological FAB classification to the most recent 2008 World Health Organization (WHO) classification that consider the morphological, immunological, cytogenetic, genetic, and clinical features. In this study we report and for the first time from Qatar the clinico-pathological features and the outcome of adult patients diagnosed as AML between January 2006 and January 2010 at Al-Amal Oncology/Hematology Center. Diagnosis was based on combined morphologic, immunophenotypic & cytogenetic studies. Seventy three patients were found to have AML accounting for 73% of all adult acute leukemias diagnosed in our center. Age was between 17 and 100 years with a median age of 40 years. Fifty five patients were males and 18 were females with a male to female ratio of 3:1. Most of the patients were non-Qatari (83.5%). The most common FAB subtype was AML –M2 (27.3%). The second most prevalent is AML-M3 (23.2%). Within this group (17.7%) were of the classical hyper granular type, while (82.3%) showed different morphological variants. Cytogenetic analysis was attempted in 64 cases. Intermediate was the most common cytogenetic risk group (64%). The most common clinical presentations were Bleeding tendency (55 patients), symptomatic anemia (52 patients) and fever (29 patients). Fifty patients were treated in our center in Qatar. Pre-treatment death occurred in 14 patients (8 patients presented with septic shock and 6 with intracranial haemorrhage). Three patients were initially palliative, 33 patients received treatment in forms of 2 inductions with Doxorubicin + Cytosine Arabinoside (DA 3+7) and 3 consolidations with Cytosine Arabinoside, pre-treatment required in 13 patients, 2 of them required leukopharesis and 11 underwent cyto-reduction by hydroxy-carbamide. Complete remission was achieved in 28 patients (85%), and 5 patients were resistant disease. Patients were followed from 60 to 1620 days with average of 429 days and Median of 330 days. As of June 2010, 24 (73%) patients are still alive, and 9 (27%) patients died. Infection was the main cause of death. In conclusion AML in Qatar have a lower median age, and an increase incidence of AML-M3 (Acute Promyelocytic) subtype that exhibit significant morphological heterogenecity (variant APL). Doxorubicin is still in use for induction in our center due to unavailability of other anthracyclines. Despite this, cardiac toxicity was not seen in our cases so far, and our complete remission and disease free survival are comparable to international data. Disclosures: No relevant conflicts of interest to declare.