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P0360 THE OCCURENCE OF THROMBOTIC THROMBOCYTOPENIC PURPURA IN A PATIENT WITH CHRONIC HEPATITIS B VIRUS INFECTION DURING TREATMENT WITH PEGYLATED INTERFERON-ALPHA-2A
Abstracts from 8th Congress of the European Federation of Internal Medicine / European Journal of Internal Medicine 20S (2009), S1–S283
S123
proved. In conclusion cerebral sinus thrombosis must be remembered in TTP cases presenting with headache.
P0359 EARLY RECOGNITION OF MALIGNANT LACTIC ACIDOSIS IN CLINICAL PRACTICE: REPORT ON 6 PATIENTS WITH HAEMATOLOGICAL MALIGNANCIES
Eva De Raes, Dominique Benoit, Pieter Depuydt, Fritz Offner, Joke Nollet, Anna Vantilborgh, Eva Steel, Luc Noens, Johan Decruyenaere. University Hospital of Ghent Belgium Background: Malignant lactic acidosis is a potentially overlooked but lifethreatening complication in patients with haematological malignancies. The aim of this study: to describe the features of six patients with malignant lactic acidosis and to discuss how its initial presentation can be differentiated from that of severe sepsis. Methods: We prospectively collected data of all consecutive patients with haematological malignancies, admitted to the Ghent University Hospital ICU (Intensive Care Unit) between 2000 and 2007. Results: Of 372 patients with haematological malignancies admitted to the ICU for life threatening complications, 58 (15.6%) presented with lactic acid levels ≥ 45 mg/dl. Six (10.3%) were diagnosed with malignant lactic acidosis. All patients with malignant lactic acidosis had high-grade lymphoblastic malignancies and were referred with a tentative diagnosis of severe sepsis or septic shock; lactic acid levels exceeded 85 mg/dL and lactate dehydrogenase (LDH) levels were at least 1785 U/L. Two patients had hypoglycaemia. All had a pronounced tachypnea. In all patients hepatic malignant involvement was suspected. Two of the six patients survived their episode thanks to the early recognition of malignant lactic acidosis and the prompt administration of chemotherapy. One patient is still alive 6 months after initiating chemotherapy. Conclusion: Malignant lactic acidosis is a rare and often rapidly fatal metabolic complication if not promptly recognized and treated. An elevated lactic acid concentration, in disproportion with the level of tissue hypoxia, together with high serum LDH are cornerstones in de diagnosis. In contrast to septic shock patients, pronounced tachypnea (Kussmaul’s breathing pattern) rather than the haemodynamic instability is prominent. Keywords: Malignant lactic acidosis, haematological malignancies, oncological emergency, differential diagnosis, sepsis
Figure 1. Peripheral blood smear of our patient with microangiopathic hemolytic anemia with marked red cell fragmentation (arrows),schistocytes and spherocytes.
Urineanalysis demostrated proteinuria and hematuria with erithrocyte casts. We diagnosed TTP and performed plasma exchange on the first hospital day then performed daily. We started 1mg/kg prednisolone and 0,5 mg/day entecavir threathment on the second hospital day. Her consciousness was gradually increase and LDH levels decreased daily (figure 2) but no significant increase was observed in her platelet counts. Starting from the day three, on the occasion of worsening renal function, and the emergence of oliguria, hemodialysis treatment was started. Her renal functions were convalescence but her LDH level started to increase and she became somnolent and then deep coma. On seventh hospitalization day she died.
Figure 2. The fluctuating course of LDH levels in our patient during her admission.
P0360 THE OCCURENCE OF THROMBOTIC THROMBOCYTOPENIC PURPURA IN A PATIENT WITH CHRONIC HEPATITIS B VIRUS INFECTION DURING TREATMENT WITH PEGYLATED INTERFERON-ALPHA-2A
Hilal Tuncer Yıimaz 1 , Irmak Sayin 1 , Ali Kemal Oguz 1 , Meltem Ayli 2 , Halil Degertekin 3 . 1 Ufuk University School of Medicine, Department of Internal Medicine; 2 Ufuk University School of Medicine, Department of Internal Medicine, Division of Hematology; 3 Ufuk University School of Medicine, Department of Internal Medicine, Division of Gastroenterology Background: Thrombotic thrombocytopenic purpura (TTP) is a life threatening microangiopathic disease, which has a sudden onset and is often fatal. TTP has many causes which includes drugs, infections, malignancies, autoimmunity, pregnancy, stem cell transplantation and mostly idiopathic. Pegylated interferon (PEG-IFN) is one of the pivotal drugs for the treatment of chronic viral hepatitis. A number of adverse reactions to PEG-IFN have been reported, including pancytopenia, psychological symptoms, renal dysfunction and immunological disorders, but it is clear that severe thrombocytopenia is one of the most serious life-threatening complication. Aims: To atract attention that TTP can found to be the underlying condition in some of thrombocytopenic cases, during the course of PEG-IFN treathment. Methods: To present a case of TTP, occurring in a patient with chronic hepatitis B virus (HBV) infection, who was under PEG-IFN treatment. Results: The patient was a 58 year-old women who received 180 ug PEG-IFN alpha-2a/week treatment for nine months for chronic hepatitis B. She admited to our hospital with complaints of abdominal pain, severe fatigue, and dark coloration of her urine developed slowly for three days. On physical examination; her temperature was 37.5°C, heart rate 90 beats/min, blood pressure 92/58 mm-Hg. She has confusion, aphasia and can make eye contact for questions asked in high volume. Her sclera and skin was icteric. Laboratory data showed that; anemia (Hb: 8,5 g/dl), thrombocytopenia (Plt: 9000/uL), elevated lactate dehydrogenase (LDH: 4958 u/L), Creatinine (3,6 mg/dl), indirect bilirubin (2,46 mg/dl). She had schistocytes on peripheral blood smear (figure 1).
Summary/conclusion: TTP is a disease characterized by a pentad of findings that include microangiopathic hemolytic anemia, thrombocytopenia, renal failure, neurologic findings, and fever. Idiopathic and drug related cases constitute the majority of TTP patients. Four TTP case reports, accusing the PEG-IFN alpha-2a therapy in patients with chronic hepatitis C virus infection, were identified in the literature. To the best of our knowledge, our case is the first which potentially implicates the use of PEG-IFN alpha-2a in the occurrence of TTP, in a patient
P0361 FATAL ANAPLASTIC LARGE CELL LYMPHOMA PRESENTING AS PAINFUL HIP
Ana Mordomo, Virgínia Mareco, Sandro Gaspar, Afonso Fernandes, Maria José Metrass, Isabel Pinheiro. Medicina Interna 2 A, Hospital Santa Maria Introduction: Anaplastic large cell lymphoma (ALCL) is an uncommon disease, accounting for 2% of all non-Hodgkin lymphomas. Among all the aggressive lymphomas, this is the type that presents the best prognosis for its good response to treatment; still, it depends on the staging at time of presentation and presence of ALK marker. Materials & methods: 52 year-old caucasian female, with a medical history of hysterectomy and bilateral ooforectomy in 2003 and nasal polyps surgery in 2007. Familial history of pulmonary and lymph node tuberculosis. In February of 2008 began anorexia with a weight loss of 20% in 2 months and was diagnosed a depression. Nine months later, without previous trauma, she noticed a painfull mass in the right hip, that extended to the lumbosacral region and right inferior limb; the pain gradually increased and was exacerbated by right lateral decubitus position, by walking and during the night, accompanied by night sweats. In December 2008, she came to the emergency department where a discal hernia was diagnosed and was medicated with diclofenac. In January of 2009 she returned to the ED with severe pain, despite multiple analgesics and was admitted in the internal medicine ward to adjust medication. The physical examination revealed a pale, apyretic patient,
S123
proved. In conclusion cerebral sinus thrombosis must be remembered in TTP cases presenting with headache.
P0359 EARLY RECOGNITION OF MALIGNANT LACTIC ACIDOSIS IN CLINICAL PRACTICE: REPORT ON 6 PATIENTS WITH HAEMATOLOGICAL MALIGNANCIES
Eva De Raes, Dominique Benoit, Pieter Depuydt, Fritz Offner, Joke Nollet, Anna Vantilborgh, Eva Steel, Luc Noens, Johan Decruyenaere. University Hospital of Ghent Belgium Background: Malignant lactic acidosis is a potentially overlooked but lifethreatening complication in patients with haematological malignancies. The aim of this study: to describe the features of six patients with malignant lactic acidosis and to discuss how its initial presentation can be differentiated from that of severe sepsis. Methods: We prospectively collected data of all consecutive patients with haematological malignancies, admitted to the Ghent University Hospital ICU (Intensive Care Unit) between 2000 and 2007. Results: Of 372 patients with haematological malignancies admitted to the ICU for life threatening complications, 58 (15.6%) presented with lactic acid levels ≥ 45 mg/dl. Six (10.3%) were diagnosed with malignant lactic acidosis. All patients with malignant lactic acidosis had high-grade lymphoblastic malignancies and were referred with a tentative diagnosis of severe sepsis or septic shock; lactic acid levels exceeded 85 mg/dL and lactate dehydrogenase (LDH) levels were at least 1785 U/L. Two patients had hypoglycaemia. All had a pronounced tachypnea. In all patients hepatic malignant involvement was suspected. Two of the six patients survived their episode thanks to the early recognition of malignant lactic acidosis and the prompt administration of chemotherapy. One patient is still alive 6 months after initiating chemotherapy. Conclusion: Malignant lactic acidosis is a rare and often rapidly fatal metabolic complication if not promptly recognized and treated. An elevated lactic acid concentration, in disproportion with the level of tissue hypoxia, together with high serum LDH are cornerstones in de diagnosis. In contrast to septic shock patients, pronounced tachypnea (Kussmaul’s breathing pattern) rather than the haemodynamic instability is prominent. Keywords: Malignant lactic acidosis, haematological malignancies, oncological emergency, differential diagnosis, sepsis
Figure 1. Peripheral blood smear of our patient with microangiopathic hemolytic anemia with marked red cell fragmentation (arrows),schistocytes and spherocytes.
Urineanalysis demostrated proteinuria and hematuria with erithrocyte casts. We diagnosed TTP and performed plasma exchange on the first hospital day then performed daily. We started 1mg/kg prednisolone and 0,5 mg/day entecavir threathment on the second hospital day. Her consciousness was gradually increase and LDH levels decreased daily (figure 2) but no significant increase was observed in her platelet counts. Starting from the day three, on the occasion of worsening renal function, and the emergence of oliguria, hemodialysis treatment was started. Her renal functions were convalescence but her LDH level started to increase and she became somnolent and then deep coma. On seventh hospitalization day she died.
Figure 2. The fluctuating course of LDH levels in our patient during her admission.
P0360 THE OCCURENCE OF THROMBOTIC THROMBOCYTOPENIC PURPURA IN A PATIENT WITH CHRONIC HEPATITIS B VIRUS INFECTION DURING TREATMENT WITH PEGYLATED INTERFERON-ALPHA-2A
Hilal Tuncer Yıimaz 1 , Irmak Sayin 1 , Ali Kemal Oguz 1 , Meltem Ayli 2 , Halil Degertekin 3 . 1 Ufuk University School of Medicine, Department of Internal Medicine; 2 Ufuk University School of Medicine, Department of Internal Medicine, Division of Hematology; 3 Ufuk University School of Medicine, Department of Internal Medicine, Division of Gastroenterology Background: Thrombotic thrombocytopenic purpura (TTP) is a life threatening microangiopathic disease, which has a sudden onset and is often fatal. TTP has many causes which includes drugs, infections, malignancies, autoimmunity, pregnancy, stem cell transplantation and mostly idiopathic. Pegylated interferon (PEG-IFN) is one of the pivotal drugs for the treatment of chronic viral hepatitis. A number of adverse reactions to PEG-IFN have been reported, including pancytopenia, psychological symptoms, renal dysfunction and immunological disorders, but it is clear that severe thrombocytopenia is one of the most serious life-threatening complication. Aims: To atract attention that TTP can found to be the underlying condition in some of thrombocytopenic cases, during the course of PEG-IFN treathment. Methods: To present a case of TTP, occurring in a patient with chronic hepatitis B virus (HBV) infection, who was under PEG-IFN treatment. Results: The patient was a 58 year-old women who received 180 ug PEG-IFN alpha-2a/week treatment for nine months for chronic hepatitis B. She admited to our hospital with complaints of abdominal pain, severe fatigue, and dark coloration of her urine developed slowly for three days. On physical examination; her temperature was 37.5°C, heart rate 90 beats/min, blood pressure 92/58 mm-Hg. She has confusion, aphasia and can make eye contact for questions asked in high volume. Her sclera and skin was icteric. Laboratory data showed that; anemia (Hb: 8,5 g/dl), thrombocytopenia (Plt: 9000/uL), elevated lactate dehydrogenase (LDH: 4958 u/L), Creatinine (3,6 mg/dl), indirect bilirubin (2,46 mg/dl). She had schistocytes on peripheral blood smear (figure 1).
Summary/conclusion: TTP is a disease characterized by a pentad of findings that include microangiopathic hemolytic anemia, thrombocytopenia, renal failure, neurologic findings, and fever. Idiopathic and drug related cases constitute the majority of TTP patients. Four TTP case reports, accusing the PEG-IFN alpha-2a therapy in patients with chronic hepatitis C virus infection, were identified in the literature. To the best of our knowledge, our case is the first which potentially implicates the use of PEG-IFN alpha-2a in the occurrence of TTP, in a patient
P0361 FATAL ANAPLASTIC LARGE CELL LYMPHOMA PRESENTING AS PAINFUL HIP
Ana Mordomo, Virgínia Mareco, Sandro Gaspar, Afonso Fernandes, Maria José Metrass, Isabel Pinheiro. Medicina Interna 2 A, Hospital Santa Maria Introduction: Anaplastic large cell lymphoma (ALCL) is an uncommon disease, accounting for 2% of all non-Hodgkin lymphomas. Among all the aggressive lymphomas, this is the type that presents the best prognosis for its good response to treatment; still, it depends on the staging at time of presentation and presence of ALK marker. Materials & methods: 52 year-old caucasian female, with a medical history of hysterectomy and bilateral ooforectomy in 2003 and nasal polyps surgery in 2007. Familial history of pulmonary and lymph node tuberculosis. In February of 2008 began anorexia with a weight loss of 20% in 2 months and was diagnosed a depression. Nine months later, without previous trauma, she noticed a painfull mass in the right hip, that extended to the lumbosacral region and right inferior limb; the pain gradually increased and was exacerbated by right lateral decubitus position, by walking and during the night, accompanied by night sweats. In December 2008, she came to the emergency department where a discal hernia was diagnosed and was medicated with diclofenac. In January of 2009 she returned to the ED with severe pain, despite multiple analgesics and was admitted in the internal medicine ward to adjust medication. The physical examination revealed a pale, apyretic patient,