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PP-071 MULTIPLE MYELOMA PRESENTING WITH CLIVUS PLASMACYTOMA
Poster Presentations – Vth International Eurasian Hematology Congress / Leukemia Research 38 S1 (2014) S1–S65
therapy. Six months later, we started the therapy of equine anti-thymocyte globulin and cyclosporin. After one year, there was still no response. We started the therapy of eltrombopag in March 2013. She has been treated by eltrombopag for sixteen months. In the last hemogram, the results were as follows: wbc: 2800 mm3 , hb: 13.5 g/dl, plt: 28.000 mm3 (in peripheral blood smear plt: 40000). Before eltrombopag therapy, she had transfusion once a week regularly. Currently, she has no transfusion during the following by eltrombopag. Furthermore, she has no symptoms of bleeding. Conclusion: Treatment with eltrombopag was associated with multilineage clinical responses in some patients with refractory aplastic anemia. This case was presented due to recovery from anemia and trombocytopenia in aplastic anemia PP-070 HEMOGLOBIN O ARAB IN AN ASYMPTOMATIC PATIENT M. Duran, M. Comert, G. Saydam. Ege University Hospital Department of Hematology Introduction: Hemoglobin O-Arab is a very rare beta globin chain mutation and is characterized by the presence of beta 121Glu >Lys (Hb O (Arab)). It has only been found among people who originate from North Africa, Northern Greece, Romania, Bulgaria and Turkey. The diagnosis of HbO (Arab) requires high-performance liquid chromatography (HPLC) on both cellulose acetate and citrate agar, since Hb O (Arab) co-migrates with Hb C at alkaline Ph. Homozygous for HbO-Arab may have a mild, asymptomatic, compensated hemolytic anemia Case: In Turkey, premarital screening for thalassemia has been normal practice for a long time. A 29-year-old male had no symptom and presented with result of HPLC, which was reported retention time between HbC and HbO and HbO level 22.1%, HbA level 70.3%, HbA2 3.2%. The peripheral blood smear and laboratory examination results were normal. In the light of these findings, we concluded with hemoglobin O Arab. Discussion: Haemoglobin O Arab is abnormal hemoglobin that is well tolerated except for heterozygous category. The heterozygote form of A O Arab and the association Hb C–Hb O Arab do not present any clinical and hematological consequences. The evolution is generally good with a long survival and no symptoms. The association of Hb S–Hb O Arab brings about a serious hemoglobinopathy, which has clinical and hematological features like the sickle-cell disease. Premarital screening aims to identify carriers of the hemoglobin disorders in order to assess the risk of having children with a severe form of disease. The couple can then choose whether or not to have an affected child. A simple blood test before marriage can easily detect carriers of haemoglobinopathies to inform couples about their chances of producing affected children and ensure they receive appropriate advice by genetic counseling.
S49
Discussion: Patients with MM present with a number of neurologic symptoms related to the involvement of the nervous system or the impact of cytokine or paraproteins on the nervous system. Brain, especially clivus involvement in MM is uncommon. Extramedullary involvement of MM has a poor prognosis. The sixth cranial nerve is the most frequent cranial nerve affected. Patients with MM and associated intracranial plasmacytoma are treated with localized radiotherapy followed by systemic chemotherapy. It is important to consider plasmacytoma in the differential diagnosis of all skull base tumors, as it is a highly radiosensitive and potentially curable disease. PP-072 NAILFOLD CAPILLAROSCOPY MAY BE A USEFUL TOOL FOR DETECTION OF MICROVASCULAR CHANGES IN PATIENTS WITH PSV S. Yolbas 1 , A. Yildirim 1 , I. Dolasik 2 , B. Uz 2 , S. Koca 1 . 1 Firat University, Faculty of Medicine, Department of Rheumatology; 2 Firat University, Faculty of Medicine, Department of Hematology Background: Nailfold capillaroscopy (NFC) is a noninvasive, inexpensive and effective technique for the evaluation of structural abnormalities of the microcirculation in the patients with Raynaud’s phenomenon and the early diagnosis of systemic sclerosis (SSc). Hematological diseases can lead to changes in shapes and numbers of peripheral blood cells and blood viscosity. Obstruction of the microcirculation and a decrease in capillary flow may cause peripheral microangiopathy and capillaroscopic changes. Objectives: The aim of the present study was to evaluate nailfold capillary changes in polycythemia vera (PSV) Method: 12 patients with PSV, 19 patients with SSc and 16 healthy controls (HC) were enrolled in the study. All participants were evaluated for capillary density, distribution and morphology, assessing features such as enlargement of the capillaries, megacapillary, tortuosity, microhaemorrhages, edema, ramified capillaries, angiogenesis and avascular areas by a NFC (Scalar, x200 magnification). Results: When compared with the HC group, some capillaroscopic findings including capillary ectasie, microhaemorrhages, angiogenesis, ramified capillaries, tortuosity, nonhomogeneous distribution, interstitial edema, Nonhomogeneous Capillary morphology, decreased capillary density, nonvisiable subpapiller venoz plexus, microthrombus, capillary dropout were more frequent in the PSV group (Table 1). But, there was no statistically significant difference between PSV and HC groups in terms of megacapillary, microaneurysms, avascular areas andenlarged loops (p>0.05, for all). The frequency of some capillaroscopic finding including capillary ectasie, microhaemorrhages, megacapillary, nonhomogeneous distribution, nonhomogeneous capillary morphology and decreased capillary density were lower in the PSV group than in the SSc group (Table 1). In the PSV group, capillary number was lower compared to the HC group; however, it was higher compared the SSc group (p<0.001 and p=0.01, respectively).
PP-071 MULTIPLE MYELOMA PRESENTING WITH CLIVUS PLASMACYTOMA M. Duran, M. Comert, H. Kiper, I. Bulgur, M. Tombuloglu. Ege University Hospital Department of Hematology Introduction: Multiple myeloma (MM) involves terminally differentiated plasma cells. MM can affect multiple organ systems; therefore, it may mimic different clinical syndromes at presentation and be found as an isolated lesion or as a part of MM. We report an extremely rare presentation of MM with clivus plasmacytoma. Case: A 66-year-old female presented with diplopia. Physical examination demonstrated 6th cranial nerve palsy. Magnetic resonance image (MRI) of the hypophysis revealed a heterogeneously enhancing expansible malign mass in clivus, which extended into Meckel’s cave. Neurosurgery was performed for resection of the mass. Mass specimen showed extensive infiltration of kappa positive plasma cells. In laboratory examination; hemoglobin 6.6 g/dl, calcium 9.7 mg/dl, creatinin: 1.43 mg/dl, kappa 141 mg/dl, erythrocyte sedimentation rate >140 mm, serum immunofixation electrophoresis kappa light chain monoclonal gammopathy was detected. Serum protein electrophoresis was normal. Skletal survey radiography showed multiple lytic lesions. Bone marrow biopsy showed diffuse (90%) plasma cells infiltration. Bortezomibe (1.3mg/m2 ), cyclophosphamide (500mg/day) and dexamethasone (40mg/day) were initiated. After two cycles control cranium MRI demonstrated residue polypoid solid particles. Whole brain cranial irradiation (30 Gy in 12 fractionated doses) was performed. After radiotherapy the patient has shown clinical improvement with disappearance of diplopia.
Conclusion: Our preliminary study documents that PSV leads to the microvascular changes including capillary dropout, microthrombus and angiogenesis, and NFC successfully identifies these changes.
therapy. Six months later, we started the therapy of equine anti-thymocyte globulin and cyclosporin. After one year, there was still no response. We started the therapy of eltrombopag in March 2013. She has been treated by eltrombopag for sixteen months. In the last hemogram, the results were as follows: wbc: 2800 mm3 , hb: 13.5 g/dl, plt: 28.000 mm3 (in peripheral blood smear plt: 40000). Before eltrombopag therapy, she had transfusion once a week regularly. Currently, she has no transfusion during the following by eltrombopag. Furthermore, she has no symptoms of bleeding. Conclusion: Treatment with eltrombopag was associated with multilineage clinical responses in some patients with refractory aplastic anemia. This case was presented due to recovery from anemia and trombocytopenia in aplastic anemia PP-070 HEMOGLOBIN O ARAB IN AN ASYMPTOMATIC PATIENT M. Duran, M. Comert, G. Saydam. Ege University Hospital Department of Hematology Introduction: Hemoglobin O-Arab is a very rare beta globin chain mutation and is characterized by the presence of beta 121Glu >Lys (Hb O (Arab)). It has only been found among people who originate from North Africa, Northern Greece, Romania, Bulgaria and Turkey. The diagnosis of HbO (Arab) requires high-performance liquid chromatography (HPLC) on both cellulose acetate and citrate agar, since Hb O (Arab) co-migrates with Hb C at alkaline Ph. Homozygous for HbO-Arab may have a mild, asymptomatic, compensated hemolytic anemia Case: In Turkey, premarital screening for thalassemia has been normal practice for a long time. A 29-year-old male had no symptom and presented with result of HPLC, which was reported retention time between HbC and HbO and HbO level 22.1%, HbA level 70.3%, HbA2 3.2%. The peripheral blood smear and laboratory examination results were normal. In the light of these findings, we concluded with hemoglobin O Arab. Discussion: Haemoglobin O Arab is abnormal hemoglobin that is well tolerated except for heterozygous category. The heterozygote form of A O Arab and the association Hb C–Hb O Arab do not present any clinical and hematological consequences. The evolution is generally good with a long survival and no symptoms. The association of Hb S–Hb O Arab brings about a serious hemoglobinopathy, which has clinical and hematological features like the sickle-cell disease. Premarital screening aims to identify carriers of the hemoglobin disorders in order to assess the risk of having children with a severe form of disease. The couple can then choose whether or not to have an affected child. A simple blood test before marriage can easily detect carriers of haemoglobinopathies to inform couples about their chances of producing affected children and ensure they receive appropriate advice by genetic counseling.
S49
Discussion: Patients with MM present with a number of neurologic symptoms related to the involvement of the nervous system or the impact of cytokine or paraproteins on the nervous system. Brain, especially clivus involvement in MM is uncommon. Extramedullary involvement of MM has a poor prognosis. The sixth cranial nerve is the most frequent cranial nerve affected. Patients with MM and associated intracranial plasmacytoma are treated with localized radiotherapy followed by systemic chemotherapy. It is important to consider plasmacytoma in the differential diagnosis of all skull base tumors, as it is a highly radiosensitive and potentially curable disease. PP-072 NAILFOLD CAPILLAROSCOPY MAY BE A USEFUL TOOL FOR DETECTION OF MICROVASCULAR CHANGES IN PATIENTS WITH PSV S. Yolbas 1 , A. Yildirim 1 , I. Dolasik 2 , B. Uz 2 , S. Koca 1 . 1 Firat University, Faculty of Medicine, Department of Rheumatology; 2 Firat University, Faculty of Medicine, Department of Hematology Background: Nailfold capillaroscopy (NFC) is a noninvasive, inexpensive and effective technique for the evaluation of structural abnormalities of the microcirculation in the patients with Raynaud’s phenomenon and the early diagnosis of systemic sclerosis (SSc). Hematological diseases can lead to changes in shapes and numbers of peripheral blood cells and blood viscosity. Obstruction of the microcirculation and a decrease in capillary flow may cause peripheral microangiopathy and capillaroscopic changes. Objectives: The aim of the present study was to evaluate nailfold capillary changes in polycythemia vera (PSV) Method: 12 patients with PSV, 19 patients with SSc and 16 healthy controls (HC) were enrolled in the study. All participants were evaluated for capillary density, distribution and morphology, assessing features such as enlargement of the capillaries, megacapillary, tortuosity, microhaemorrhages, edema, ramified capillaries, angiogenesis and avascular areas by a NFC (Scalar, x200 magnification). Results: When compared with the HC group, some capillaroscopic findings including capillary ectasie, microhaemorrhages, angiogenesis, ramified capillaries, tortuosity, nonhomogeneous distribution, interstitial edema, Nonhomogeneous Capillary morphology, decreased capillary density, nonvisiable subpapiller venoz plexus, microthrombus, capillary dropout were more frequent in the PSV group (Table 1). But, there was no statistically significant difference between PSV and HC groups in terms of megacapillary, microaneurysms, avascular areas andenlarged loops (p>0.05, for all). The frequency of some capillaroscopic finding including capillary ectasie, microhaemorrhages, megacapillary, nonhomogeneous distribution, nonhomogeneous capillary morphology and decreased capillary density were lower in the PSV group than in the SSc group (Table 1). In the PSV group, capillary number was lower compared to the HC group; however, it was higher compared the SSc group (p<0.001 and p=0.01, respectively).
PP-071 MULTIPLE MYELOMA PRESENTING WITH CLIVUS PLASMACYTOMA M. Duran, M. Comert, H. Kiper, I. Bulgur, M. Tombuloglu. Ege University Hospital Department of Hematology Introduction: Multiple myeloma (MM) involves terminally differentiated plasma cells. MM can affect multiple organ systems; therefore, it may mimic different clinical syndromes at presentation and be found as an isolated lesion or as a part of MM. We report an extremely rare presentation of MM with clivus plasmacytoma. Case: A 66-year-old female presented with diplopia. Physical examination demonstrated 6th cranial nerve palsy. Magnetic resonance image (MRI) of the hypophysis revealed a heterogeneously enhancing expansible malign mass in clivus, which extended into Meckel’s cave. Neurosurgery was performed for resection of the mass. Mass specimen showed extensive infiltration of kappa positive plasma cells. In laboratory examination; hemoglobin 6.6 g/dl, calcium 9.7 mg/dl, creatinin: 1.43 mg/dl, kappa 141 mg/dl, erythrocyte sedimentation rate >140 mm, serum immunofixation electrophoresis kappa light chain monoclonal gammopathy was detected. Serum protein electrophoresis was normal. Skletal survey radiography showed multiple lytic lesions. Bone marrow biopsy showed diffuse (90%) plasma cells infiltration. Bortezomibe (1.3mg/m2 ), cyclophosphamide (500mg/day) and dexamethasone (40mg/day) were initiated. After two cycles control cranium MRI demonstrated residue polypoid solid particles. Whole brain cranial irradiation (30 Gy in 12 fractionated doses) was performed. After radiotherapy the patient has shown clinical improvement with disappearance of diplopia.
Conclusion: Our preliminary study documents that PSV leads to the microvascular changes including capillary dropout, microthrombus and angiogenesis, and NFC successfully identifies these changes.