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C0536: Digital Microthrombosis in Systemic Sclerosis: Think About Essential Thrombocythemia
POSTERS / Thrombosis Research 133S3 (2014) S35–S123
Case presentation: A 26-year-old man presented with pain in the right leg. The patient’s medical history included a diagnosis of brucellosis in our hospital where he had presented with complaints of weakness and fever. Peripheral venous Doppler ultrasound showed DVT and patient was treated with anticoagulants. The patient was discharged with warfarin therapy and anti-brucellosis treatment. Conclusions: Although rare, some infectious agents may cause vascular pathologies. Patients presenting with symptoms of DVT or similar vascular pathologies should be assessed for infectious agents particularly in those coming from Brucella-endemic areas. C0466 TOTAL ABDOMINAL HYSTERECTOMY AND POSTOPERATIVE BLEEDING DIATHESIS: A CASE REPORT T. Vucemilo1 , M. Puljiz1 , I. Alvir1 , I. Mamic1 , M. Skoko1 . 1 University Hospital Sisters of Charity University Hospital for Tumors. Transfusion, Zagreb, Croatia Background: Uterine myoma is the most common benign genital tumor among women of reproductive age. Total abdominal hysterectomy (TAH) is the most common type of hysterectomy. The average blood loss during TAH is approximately 300ml. Surgical blood loss that requires a blood transfusion is usually defined as intraoperative hemorrhage. Intraoperative hemorrhage has been reported in 1% to 2% of hysterectomy studies. Methods: We present a case of TAH complicated with bleeding diathesis and blood loss more than 2500 ml. A 42-year-old woman 3-para had a five year history uterine myoma. Her past medical history was free of any medical problems. She was found to be anemic with Hg of 6.3 g/dl. Four days before surgery she had 600 ml packed red blood cells transfused. Before surgery her Hg was 10 g/dl. PLT 233x/l Coagulation parameters were normal: PT 12.6 s, APTT 29 s, TT 15 s, Fibrinogen 4.2 g/l. She underwent open myomectomy via a low transverse incision when the uterus found to be enlarged to the size of a 12-week pregnancy. The estimated blood loss was 300 ml and there were no complications. The patient was hemodinamically stable until the end of surgery. Three to four hours postoperatively she became dyspneic. She was hypotensive and tachycardic. Her Hg dropped to 5.4 g/l. She had remarkably prolonged coagulation parameters PT 23.5 s, INR 2.0, APTT 69 s, TT 27 s, Fibrinogen 1.0 g/l, PLT 60. Ultrasonography revealed free fluid filling the pelvis in lower abdomen. A diagnosis of intrabdominal bleeding was set and she underwent immediate laparotomy with appropriate resuscitation with i.v. fluid, blood, FFP, cryoprecipitate and ciclokapron. Results: At laparotomy an estimated 2000 ml of blood was found in the peritoneal cavity, and there was no evidence of active arterial bleeding. The total estimated blood loss during surgery was 2500 ml and patient received 2100 ml packed red blood cells, two unit FFP, two pools platelets, two dose cryoprecipitate 850 AHF, ciclokapron 1500 mg. Under supplementation of blood components subsequent laboratory finding improved with stabile vital sign and a satisfactory recovery. Conclusions: Bleeding diathesis can be caused by many solid tumors but TAH induced bleeding diathesis is rare. Recently published data demonstrates that women presented with menorrhagia frequently report an additional history of bleeding after surgery. We need additional efforts to develop hematological screening tools within women presenting with menorrhagia.
S57
C0536 DIGITAL MICROTHROMBOSIS IN SYSTEMIC SCLEROSIS: THINK ABOUT ESSENTIAL THROMBOCYTHEMIA F. Ajili1 , A. Tliba1 , N. Boussetta1 , H. Ghedira1 , I. Gharsallah1 , L. Metoui1 , B. Louzir1 , N. ben Abdelhafidh1 , S. Othmani1 . 1 Military Hospital in Tunis. Internal Medicine. Tunis, Tunisia Background: Digital microthrombosis are a well-known problem in patients with systemic sclerosis. Essential thrombocytosis is another cause of this disease. It causes digital ulcers which increase impairment of hand function. Methods: We report clinical charts of a female 68 year old patient with a rare association of essential thrombocythemia and systemic sclerosis. Results: A 68 year old women presented with a 2 week history of pain and bilateral bluish discoloration of the finger tips and toes, epigastric pain, post prandial vomiting, heartburn and dysphagia. Physical examination showed proximal skin sclerosis, thin skin, less hair, bilateral and symmetrical sclerodactily and pulp ulcers of ten fingers and the big toes. Abnormality in complementary investigations were platelet count of 634000 elements/mm3 in blood test, interstitel syndrome in chest radiography and resorption of phalangeal tufts of several fingers in hand radiographs. Then the diagnosis of systemic sclerosis was confirmed. The clinical outcome, after treatment with calcium channel blockers and colchicine, was marked by the extent of digital ulcers. On the other hand, the thrombocytosis was compounded. A bone marrow biopsy was done and concluded at a megakaryocytic lineage hyperplasia, and presence of JAk 2 mutation in molecular biology which is compatible with the diagnosis of essential thrombocythemia. The patient started a treatment based on hydroxyurea (HYDREA) with a good clinical evolution and her platelet count was of 219000 elements/mm3. Conclusions: The association of systemic sclerosis and essential thromcythemia increase the gravity of digital thrombocytosis and ulcers. Their management in this particular case requires a multimodal approach using a combination of pharmacological and local treatments. C0540 A PEDIATRIC CASE OF ACUTE LYMPHOBLASTIC LEUKEMIA WITH MULTIPLE SINUS THROMBOSIS AND DEEP VEIN THROMBOSIS Y. Kilinc1 , D. Ay Tuncel1 , A. Tunc1 . 1 Cukurova University Pediatric Hematology, Turkey Background: Thrombosis is one of the common complications of leukemia related with disease or treatment. In this report, a case of acute lymphoblastic leukemia with cerebral sinus and deep vein thrombosis was presented. Methods: Case: 13 year old, male patient with shortness of breath and pallor is recoursed to Pediatric Hematology Clinic. On physical exam he was pale, has tachypnea and his spleen was palpated 8 cm below left costal margin and liver was 10 cm. Neurological examination was normal. On X-ray scanning, he has wide mediastinum. On his laboratory examination; analysis of the peripheral blood smear revealed lymphoblasts. He has diagnosed Acute Lymphoblastic Leukemia by bone marrow aspiration plus flow cytometry. BFM ALL chemotherapy protocol was begun. Evaluation of the patient, he was included in T-ALL HRG group. During the protocol 1 phase 1 chemotherapy, after a sudden headache, convulsion occurred. On cerebral MRI scanning, thrombus was demonstrated and occluded dural sinus. Searching for a thrombophilic state, homosistein was found high. During evaluation of the patient, deep vein thrombus at the lower extremity was also seen. Enoxaparin treatment was begun. Results: The patient is still under treatment of Leukemia but enoxaparin treatment was discontinued because of bleeding complications.
Case presentation: A 26-year-old man presented with pain in the right leg. The patient’s medical history included a diagnosis of brucellosis in our hospital where he had presented with complaints of weakness and fever. Peripheral venous Doppler ultrasound showed DVT and patient was treated with anticoagulants. The patient was discharged with warfarin therapy and anti-brucellosis treatment. Conclusions: Although rare, some infectious agents may cause vascular pathologies. Patients presenting with symptoms of DVT or similar vascular pathologies should be assessed for infectious agents particularly in those coming from Brucella-endemic areas. C0466 TOTAL ABDOMINAL HYSTERECTOMY AND POSTOPERATIVE BLEEDING DIATHESIS: A CASE REPORT T. Vucemilo1 , M. Puljiz1 , I. Alvir1 , I. Mamic1 , M. Skoko1 . 1 University Hospital Sisters of Charity University Hospital for Tumors. Transfusion, Zagreb, Croatia Background: Uterine myoma is the most common benign genital tumor among women of reproductive age. Total abdominal hysterectomy (TAH) is the most common type of hysterectomy. The average blood loss during TAH is approximately 300ml. Surgical blood loss that requires a blood transfusion is usually defined as intraoperative hemorrhage. Intraoperative hemorrhage has been reported in 1% to 2% of hysterectomy studies. Methods: We present a case of TAH complicated with bleeding diathesis and blood loss more than 2500 ml. A 42-year-old woman 3-para had a five year history uterine myoma. Her past medical history was free of any medical problems. She was found to be anemic with Hg of 6.3 g/dl. Four days before surgery she had 600 ml packed red blood cells transfused. Before surgery her Hg was 10 g/dl. PLT 233x/l Coagulation parameters were normal: PT 12.6 s, APTT 29 s, TT 15 s, Fibrinogen 4.2 g/l. She underwent open myomectomy via a low transverse incision when the uterus found to be enlarged to the size of a 12-week pregnancy. The estimated blood loss was 300 ml and there were no complications. The patient was hemodinamically stable until the end of surgery. Three to four hours postoperatively she became dyspneic. She was hypotensive and tachycardic. Her Hg dropped to 5.4 g/l. She had remarkably prolonged coagulation parameters PT 23.5 s, INR 2.0, APTT 69 s, TT 27 s, Fibrinogen 1.0 g/l, PLT 60. Ultrasonography revealed free fluid filling the pelvis in lower abdomen. A diagnosis of intrabdominal bleeding was set and she underwent immediate laparotomy with appropriate resuscitation with i.v. fluid, blood, FFP, cryoprecipitate and ciclokapron. Results: At laparotomy an estimated 2000 ml of blood was found in the peritoneal cavity, and there was no evidence of active arterial bleeding. The total estimated blood loss during surgery was 2500 ml and patient received 2100 ml packed red blood cells, two unit FFP, two pools platelets, two dose cryoprecipitate 850 AHF, ciclokapron 1500 mg. Under supplementation of blood components subsequent laboratory finding improved with stabile vital sign and a satisfactory recovery. Conclusions: Bleeding diathesis can be caused by many solid tumors but TAH induced bleeding diathesis is rare. Recently published data demonstrates that women presented with menorrhagia frequently report an additional history of bleeding after surgery. We need additional efforts to develop hematological screening tools within women presenting with menorrhagia.
S57
C0536 DIGITAL MICROTHROMBOSIS IN SYSTEMIC SCLEROSIS: THINK ABOUT ESSENTIAL THROMBOCYTHEMIA F. Ajili1 , A. Tliba1 , N. Boussetta1 , H. Ghedira1 , I. Gharsallah1 , L. Metoui1 , B. Louzir1 , N. ben Abdelhafidh1 , S. Othmani1 . 1 Military Hospital in Tunis. Internal Medicine. Tunis, Tunisia Background: Digital microthrombosis are a well-known problem in patients with systemic sclerosis. Essential thrombocytosis is another cause of this disease. It causes digital ulcers which increase impairment of hand function. Methods: We report clinical charts of a female 68 year old patient with a rare association of essential thrombocythemia and systemic sclerosis. Results: A 68 year old women presented with a 2 week history of pain and bilateral bluish discoloration of the finger tips and toes, epigastric pain, post prandial vomiting, heartburn and dysphagia. Physical examination showed proximal skin sclerosis, thin skin, less hair, bilateral and symmetrical sclerodactily and pulp ulcers of ten fingers and the big toes. Abnormality in complementary investigations were platelet count of 634000 elements/mm3 in blood test, interstitel syndrome in chest radiography and resorption of phalangeal tufts of several fingers in hand radiographs. Then the diagnosis of systemic sclerosis was confirmed. The clinical outcome, after treatment with calcium channel blockers and colchicine, was marked by the extent of digital ulcers. On the other hand, the thrombocytosis was compounded. A bone marrow biopsy was done and concluded at a megakaryocytic lineage hyperplasia, and presence of JAk 2 mutation in molecular biology which is compatible with the diagnosis of essential thrombocythemia. The patient started a treatment based on hydroxyurea (HYDREA) with a good clinical evolution and her platelet count was of 219000 elements/mm3. Conclusions: The association of systemic sclerosis and essential thromcythemia increase the gravity of digital thrombocytosis and ulcers. Their management in this particular case requires a multimodal approach using a combination of pharmacological and local treatments. C0540 A PEDIATRIC CASE OF ACUTE LYMPHOBLASTIC LEUKEMIA WITH MULTIPLE SINUS THROMBOSIS AND DEEP VEIN THROMBOSIS Y. Kilinc1 , D. Ay Tuncel1 , A. Tunc1 . 1 Cukurova University Pediatric Hematology, Turkey Background: Thrombosis is one of the common complications of leukemia related with disease or treatment. In this report, a case of acute lymphoblastic leukemia with cerebral sinus and deep vein thrombosis was presented. Methods: Case: 13 year old, male patient with shortness of breath and pallor is recoursed to Pediatric Hematology Clinic. On physical exam he was pale, has tachypnea and his spleen was palpated 8 cm below left costal margin and liver was 10 cm. Neurological examination was normal. On X-ray scanning, he has wide mediastinum. On his laboratory examination; analysis of the peripheral blood smear revealed lymphoblasts. He has diagnosed Acute Lymphoblastic Leukemia by bone marrow aspiration plus flow cytometry. BFM ALL chemotherapy protocol was begun. Evaluation of the patient, he was included in T-ALL HRG group. During the protocol 1 phase 1 chemotherapy, after a sudden headache, convulsion occurred. On cerebral MRI scanning, thrombus was demonstrated and occluded dural sinus. Searching for a thrombophilic state, homosistein was found high. During evaluation of the patient, deep vein thrombus at the lower extremity was also seen. Enoxaparin treatment was begun. Results: The patient is still under treatment of Leukemia but enoxaparin treatment was discontinued because of bleeding complications.