P0361 FATAL ANAPLASTIC LARGE CELL LYMPHOMA PRESENTING AS PAINFUL HIP

Abstracts from 8th Congress of the European Federation of Internal Medicine / European Journal of Internal Medicine 20S (2009), S1–S283

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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,

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Abstracts from 8th Congress of the European Federation of Internal Medicine / European Journal of Internal Medicine 20S (2009), S1–S283

sweating, with painful fusiform tumor in the right hip, about 12x15cm. Pelvis x-ray revealed alterations of bone density in the right iliac. Abdomino-pelvic ultra-sound showed iliac, lumbo aortic and retrocrural lymphadenopathies. Renal, pelvic, thyroid and breast ultrasound were normal. Laboratory revealed increased LDH (1 372 U/L) and anemia (Hb 11.8 g/dL). Further evaluation showed thrombocytopenia (18 000) and rising values of LDH that reached 4 404U/L. Protein electrophoresis, beta 2 microglobulin and anti-HIV 1 and 2 were negative. Myelogram revealed hypercellular bone marrow with invasion by atypical cells. A CT revealed multiple iliac, retroperitoneal and supraclavicular adenopathies; lytic lesion in the left scapula; lytic mass in the right iliac bone about 12x13 cm, destroying the iliac bone, sacrum and right sacroiliac joint and invading the right gluteus muscle. Histology of CT-guided biopsy of the lesion revealed a ALCL, with CD30, CD4, ALK and EMA positive markers. The final diagnosis was systemic anaplastic lymphoma, stage IV-B, with bone marrow and muscle skeletal involvement. She was medicated with cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP chemotherapy)and died of sepsis 2 weeks later. Discussion & conclusion: ALK-positive anaplastic large-cell lymphomas have a more favorable prognosis, but staging is also a very important prognostic factor. It is therefore extremely important an early diagnosis. Unfortunately,in this particular case, the patient had a late diagnosis and did succumb to overwhelming infection before the result of chemotherapy could be evaluated. Keywords: anaplastic large cell lymphoma

P0362 INAPPROPRIATE ADH SECRETION SYNDROME IN A CHRONIC LYMPHOCYTIC LEUKEMIA CASE WITH CENTRAL NERVOUS SYSTEM INVOLVEMENT

Seyit Mehmet Kayacan, Esra Süheda ¸ Hatipo˘glu, Abdullah Özkök, Sezai Vatansever, Aytaç Karada˘g, Ahmet Bilge Sözen, Kerim Güler. Istanbul University, Istanbul Medical Faculty, Department of Internal Medicine, Istanbul, Turkey Central nervous system (CNS) involvement is a rare complication of chronic lymphocytic leukemia (CLL). Extramedullary tissue infiltration such as liver, spleen and lymph nodes is seen in late stages of the diasese. CNS involvement is seen only in severely aggresive CLL forms. In this case report, a terminal CLL case with CNS involvement and inappriopriate ADH secretion syndrome who has meningitis symptoms and is refractory to treatment is presented. Case: A 49 years old female patient who was diagnosed as CLL and did not tolarete chemotherapy because of cytopenia applied with headache and high fever. We learned that she was treated for febrile neutropenia before and no pathology was detected on her cranial CT. She was agitated and had meaningless speaches, her place-time orientation was imperfect. Fever (38.8 oC) and neck stifness were found. There was not a focus of infection. No pathology could be found in her urine examination and chest x-ray. Another cranial CT was obtained and no pathology could be found. Neutrophil 237/μl and lymphocyte 35/μl were found in the cerebrospinal fluid. In CSF, glucose was 23 mg/dl and total protein was 2770 mg/l. By gram staining abundant numbers of PNLs were seen. Any microorganisms or acid fast bacillus (AFB) were not seen. Cephtriaxone 2×2 gr iv was started. Her blood, urine and CSF cultures were sterile. Tuberculosis bacillus did not grow in CSF. Serum Na of the patient was 122 mmol/L. She was not using any diuretics. Her urine Na level was 59 mmol/l. Her blood osmolarity was 287 mosm/kg and urine osmolarity was 400 mosm/kg. Treatment was started with the diagnosis of acute symptomatic hyponatremia dependent on inappriopriate ADH secretion syndrome. After fluid restriction and 3% NaCl was infused. After 24 hours, serum Na level reached to the level of 130 mmol/L. Her consciousness was a little bit better. Her orientation was complete. However on the fourth day of antibiotic treatment her fever was still high and she still had neck stiffness. Lumber punction was done again. Lymphocytes 2790/μl were counted in CSF. The cultures were again sterile. In cytologic analysis CD3 (+) and CD20 (+) small lymphocytes were seen. By flow-cytometric study of CSF it was demonstarated that CSF infiltration was consistent with CLL involvement. Ongoing high fever and neck stiffness without a demonstrated infectious focus also supported this finding. Cephtriaxone treatment was completed to 14 days. It was considered that high fever and neck stiffness were present because of the CNS involvement of CLL. The patient was diagnosed as terminal CLL and was not accepted as a suitable candidate for chemotherapy. She was observed with symptomatic treatment and she was lost in third week. As a result, we report this case to remind the possibility of CNS involvement and inappriopriate ADH secretion in a CLL patient presenting with high fever, neck stiffness, unconsciousness and hyponatremia.

P0363 RELATIVE POLYCYTHEMIA AND THROMBO-EMBOLISM; RECOGNITION AND MANAGEMENT

Muhammad Muzaffar Mahmood, Gamal Alfitori, Satyanarayana Varma Sagi, Keith Myers. Prince Charles Hospital, Merthyr Tydfil, South Glamorgan, UK Introduction: Relative Polycythemia is defined as raised hemoglobin concentration with a normal red cell mass and is mostly associated with acute hypoxia, smoking, obesity, alcohol and diuretics. Relative polycythemia and thrombotic events have been linked previously in various case reports. However, unlike absolute polycythemia, the true risk of thromboembolism is not well known in this group. It is also unknown that how patients with relative polycythemia are managed after their hospital presentation. Objectives: The aim of our study was a) to find the incidence of thrombotic events in patients with relative polycythemia presenting to Medical Admission Unit (MAU) and Accident &Emergency (A&E) department b) record their subsequent management. Materials & methods: Patients with relative polycythemia presenting to MAU and A&E during a six years period (2000-2005) were identified from Hematology lab database. Case records were reviewed to identify causes of relative polycythemia, instances of thromboembolism (both arterial and/or venous) and any additional risk factors for thromboembolism in these patients. Subsequent management of all patients with relative polycythemia including referral to hematology, if needed, was also recorded. Results: 64 patients were identified (males 62, Age range 19-39 years). Presenting conditions were drug overdose (16), alcohol intoxication, pneumonia, atrial fibrillation, seizure, chest pain, diabetic ketoacidosis and trauma. Only 8 patients were clinically dehydrated, while Alcohol intake was reported in all patients with two-thirds admitting excessive alcohol intake. 53 patients were smokers (all with normal Oxygen saturations). 8 instances (12.5%) of thromboembolism were identified (5 Venous, 2 Cerebrovascular and 1 coronary) in these patients during the study period, none of these were recorded in patients who were dehydrated. Additional risk factors for thromboembolism were identified as follows; diabetes and smoking in 1 patient with CVA, recent immobility in 1 patient with VTE, smoking in 1 patient with coronary artery disease while 3 patients with VTE had smoking history. None of VTE patients had a history of intra-venous drug use. Hemoglobin concentration normalized after rehydration in the 8 dehydrated patients. In 19 patients follow-up (with hematology referral in a majority) was arranged while no measures were taken in rest of the patients. Only 5 of these 19 patients attended follow-up appointments. Discussion & conclusion: Smoking and heavy alcohol intake are associated with relative polycythemia and there is a higher risk of Thromoboembolism in such patients. This is particularly important as these patients are mostly younger than 40 years of age, a group in which spontaneous Thrombotic events are rare. Major obstacles to proper management of such patients are insufficient identification by health professionals and poor patient compliance with follow-up. Keywords: polycythemia; thromboemboli

P0364 HYPERERYTHROBLASTOSIS IN A CASE WITH PYRIMIDINE 5’-NUCLEOTIDASE TO WHOM SPLENECTOMY WAS PERFORMED BEFORE

Seyit Mehmet Kayacan, Ömer Celal Elçio˘glu, Esra Süheda ¸ Hatipo˘glu, Abdullah Özkök, Aytaç Karada˘g, Sezai Vatansever, Ahmet Bilge Sözen, Kerim Güler. Istanbul University, Istanbul Medical Faculty, Department of Internal Medicine, Istanbul, Turkey Pyrimidine 5’-nucleotidase is an enzyme that cleaves a phosphate group from pyrimidine ribonucleoside monophosphates (cytidine 5’-monophosphate or UMP). The hereditary deficiency of the enzyme causes a hemolytic anemia. Prominent erythroblastosis in peripheral blood is an expected finding in ongoing hemolysis after splenectomy. Cases in which erythroblast numbers reached 100000/μl in peripheral blood. Herein we report a case to whom splenectomy was performed according to the diagnosis of autoimmune hemolytic anemia but was then diagnosed as pyrimidine 5’-nucleotidase enzyme deficiency and in whom after splenectomy erythroblast number increased above 250000/μl. Case: A-31 years old female patient who applied with fatigue. She had anemia before 14 years at time of her pregnancy. During and after her pregnancy she had multiple blood transfusions and later she had no complaints. Five years ago, splenectomy was performed for coombs positive hemolytic anemia. Ten days after splenectomy, left portal vein thrombosis was detected. She used coumadin for 4 months and then she stopped. Direct and indirect coombs