Lupus anticoagulant-hypoprothrombinemia syndrome: report of two cases and review of the literature.

Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare acquired disorder caused by prothrombin antibodies. The disease is most common in the pediatric age group (<16 years), and more prevalent in women. There are well-established clinical diseases associated with LA-HPS, most notably systemic lupus erythematosus (SLE) and viral infections. The clinical manifestation of LA-HPS varies greatly in severity and it may cause severe life-threatening bleeding diathesis. LA-HPS is to be suspected when a patient presents with bleeding and a prolonged activated partial thromboplastin and prothrombin time, in combination with a lupus anticoagulant. The diagnosis is confirmed in the laboratory by identification of reduced prothrombin levels. There are no standardized recommendations for treatment of the hemorrhage associated with the syndrome; corticosteroids are used as first-line treatment. This review summarizes what is currently known about the pathogenesis, clinical features, diagnosis, treatment and prognosis of LA-HPS, and presents two case reports.

Acquired hemophilia: a case report and review of the literature.

Acquired hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against clotting factor VIII (FVIII). FVIII autoantibody is characterized as polyclonal immunoglobulin G directed against the FVIII procoagulant activity. This disease occurs most commonly in the elderly population and with preponderance of men in nonpregnancy-related AHA. There are well-established clinical associations with AHA such as malignancy, other autoimmune diseases and pregnancy. However, up to 50% of reported cases remain idiopathic. The clinical manifestation of AHA includes mostly spontaneous hemorrhages into skin, muscles and soft tissues, or mucous membranes. AHA should be suspected when a patient with no previous history of bleeding presents with bleeding and an unexplained prolonged activated partial thromboplastin time. The diagnosis is confirmed in the laboratory by the subsequent identification of reduced FVIII levels and FVIII inhibitor titration. There is a high mortality, making prompt diagnosis and treatment vitally important. The principles of treatment consist in controlling the bleeding and eradicating the inhibitor. Because of the overall high relapse rate (15-33%), it is also recommended to follow up these patients. The review summarizes what is currently known about the epidemiology, pathogenesis, clinical features, diagnosis, treatment and prognosis of AHA and starts with a case report.

Early recognition of malignant lactic acidosis in clinical practice: report on 6 patients with haematological malignancies.

BACKGROUND: Malignant lactic acidosis is a potentially overlooked but life-threatening complication in patients with haematological malignancies. The aim of this study is 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 Intensive Care Unit (ICU) between 2000 and 2007. RESULTS: Of 372 patients with haematological malignancies admitted to the ICU for life- threatening complications, 58 presented with lactic acid levels > or = 5 mmol/L. Six 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 9.45 mmol/L and lactate dehydrogenase (LDH) levels were at least 1785 U/L. Two patients had hypoglycaemia. All had a pronounced polypnea. 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 was 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 the diagnosis. In contrast to septic shock patients, pronounced polypnea (Kussmaul's breathing pattern) rather than the haemodynamic instability is prominent.

Myeloma of the central nervous system: report of a single center case series.

BACKGROUND: Neurologic manifestations often complicate the course of multiple myeloma, but direct involvement of the central nervous system (CNS) is rare. OBJECTIVE: To describe the clinical course, neurological symptoms, laboratory findings and imaging of patients with CNS myeloma. This additional information may contribute to better recognition and more effective management of this complication in the future. METHODS: We retrospectively identified 6 MM patients with CNS involvement diagnosed at our centre between April 2003 and April 2009. The clinical, biochemical and imaging data were collected and compared with previously reported cases. RESULTS: At time of diagnosis of CNS myeloma, 3 patients had progressive disease and 3 were in good partial remission. The presenting symptoms included diplopia, vision loss, extremity weakness and paresis. All cases showed one or more features of aggressive disease at diagnosis, including high tumour burden, plasmablastic morphology and high-risk cytogenetic abnormalities. Prognosis was poor with a median survival of 4.8 months. CONCLUSION: CNS myeloma should be considered in patients with aggressive MM and unexplained neurological symptoms. The prognosis is poor despite intensive therapy.