Kikuchi's lymphadenopathy: a relatively rare but important cause of lymphadenopathy in Greece, potentially associated with the antiphospholipid syndrome.

Kikuchi-Fujimoto disease is a form of reactive lymphadenopathy, which was firstly described in Japan, but is uncommon in the Western world. We retrospectively reviewed the medical records of nine cases of adult or adolescent Kikuchi's disease diagnosed in a single Haematology Unit in Athens, Greece between 1990 and 2006. The median age of the patients was 25 years (14-40) and 8/9 were females. All patients presented with cervical lymphadenopathy sparing the supraclavicular fossa; one had associated axillary lymphadenopathy, seven had fever and two were asymptomatic. The median duration of lymphadenopathy before presentation was 30 days (10-45). Just palpable splenomegaly was recorded in three patients. The median value of the maximal lymph node diameter was 2 cm (1-5) and only 1/9 had nodes >2 cm in their largest diameter. Lymphadenopathy was tender in two patients; hard nodes were observed in three patients. The median leukocyte count was 4.7 x 10(9)/l (2.2-4.9) with a normal differential in 7/9 patients. No infectious agent could be demonstrated. One patient had clinical and laboratory evidence of primary antiphospholipid syndrome (APLS). In conclusion, Kikuchi's disease represents a rare but important diagnostic possibility for patients presenting with lymphadenopathy in Greece and other western countries. In this setting, autoimmune disorders, mainly lupus and APLS, should be considered and excluded by the appropriate laboratory work-up.

Primary adult-onset macrophage activation syndrome with multisystemic tissue phagocytosis.

The histiocyte disorders are divided into the following 3 categories according to the specific lineage of the histiocytes involved and their biological behavior: the dendritic cell-related disorders, which include Langerhans cell histiocytosis and dermal dendrocyte disorders; the macrophage cell disorders, hemophagocytic lymphohistiocytosis being the main entity; and the malignant histiocyte disorders. We present a case of a 36-year-old woman who was referred to our hospital because of fever of unknown origin, lethargy, anemia, and impaired hepatic function. Following a thorough investigation, we diagnosed extensive histiocyte-mediated phagocytosis in many areas (skin, liver, bone marrow), without any identifiable cause. The disease was controlled by immunosuppressive therapy, and the patient remains in complete remission. This case supports the concept of idiopathic generalized histiocyte activation as a distinct entity; this putative disease entity produces massive phagocytosis, regardless of the type of histiocyte differentiation. Similar cases necessitate further study for classification and management.

Erythropoietin abuse and erythropoietin gene doping: detection strategies in the genomic era.

The administration of recombinant human erythropoietin (rhEPO) increases the maximum oxygen consumption capacity, and is therefore abused as a doping method in endurance sports. The detection of erythropoietin (EPO) abuse is based on direct pharmacological and indirect haematological approaches, both of which have several limitations. In addition, current detection methods cannot cope with the emerging doping strategies of EPO mimicry, analogues and gene doping, and thus novel detection strategies are urgently needed. Direct detection methods for EPO misuse can be either pharmacological approaches that identify exogenous substances based on their physicochemical properties, or molecular methods that recognise EPO transgenes or gene transfer vectors. Since direct detection with molecular methods requires invasive procedures, it is not appropriate for routine screening of large numbers of athletes. In contrast, novel indirect methods based on haematological and/or molecular profiling could be better suited as screening tools, and athletes who are suspect of doping would then be submitted to direct pharmacological and molecular tests. This article reviews the current state of the EPO doping field, discusses available detection methods and their shortcomings, outlines emerging pharmaceutical and genetic technologies in EPO misuse, and proposes potential directions for the development of novel detection strategies.

Guillain-Barre syndrome presenting with sensory disturbance following a herpes virus infection: a case report.

INTRODUCTION: We present a case of an unusual clinical manifestation of Guillain-Barre syndrome following a pre-existing herpes virus infection. Although there have been several reports describing the co-existence of herpes virus infection and Guillain-Barre syndrome, we undertook a more in-depth study of the cross-reactivity between herpes viruses and recommend a follow-up study based on serology tests. CASE PRESENTATION: A 39-year-old healthy Caucasian man with Guillain-Barre syndrome presented to our facility initially with sensory disturbance, followed by an atypical descending pattern of clinical progression. On physical examination, our patient showed hot and cold temperature sensory disturbance under the T4 vertebrae level, symmetrically diminished muscle power mainly to his lower limbs, blurred vision, a loss of taste and paresis and diminished reflexes of his lower limbs. Serology test results for common viruses on hospital admission were positive for cytomegalovirus immunoglobulin M, cytomegalovirus immunoglobulin G, herpes simplex virus immunoglobulin M, herpes simplex virus immunoglobulin G, Epstein-Barr virus immunoglobulin M, and varicella zoster virus immunoglobulin G, borderline for Epstein-Barr virus immunoglobulin G and negative for varicella zoster virus immunoglobulin M. At one month after hospital admission his test results were positive for cytomegalovirus immunoglobulin M, cytomegalovirus immunoglobulin G, herpes simplex virus immunoglobulin G, Epstein-Barr virus immunoglobulin G, varicella zoster virus immunoglobulin G, borderline for herpes simplex virus immunoglobulin M and negative for Epstein-Barr virus immunoglobulin M and varicella zoster virus immunoglobulin M. At his six month follow-up, tests were positive for cytomegalovirus immunoglobulin G, herpes simplex virus immunoglobulin M, herpes simplex virus immunoglobulin G, Epstein-Barr virus immunoglobulin G and varicella zoster virus immunoglobulin G and negative for cytomegalovirus immunoglobulin M, Epstein-Barr virus immunoglobulin M and varicella zoster virus immunoglobulin M. CONCLUSIONS: The clinical manifestation of Guillain-Barre syndrome in our patient followed a combined herpes virus infection. The cross-reactivity between these human herpes viruses may have a pathogenic as well as evolutionary significance. Our patient showed seroconversion at an early stage of Epstein-Barr virus immunoglobulin M to immunoglobulin G antibodies, suggesting that Epstein-Barr virus might have been the cause of this syndrome. Even if this case is not the first of its kind to be reported, it may contribute to a better understanding of the disease and the cross-reaction mechanisms of herpes virus infections. This case report may have a broader clinical impact across more than one area of medicine, suggesting that cooperation between different specialties is always in the patient's best interest.

Staphylococcus aureus abscess of the spleen in a beta-thalassemia patient.

Splenic abscesses are rare among abdominal abscesses. We present a case of splenic abscess due to Staphylococcus aureus in a beta-thalassemia major patient. Such a complication may not be coincidental, as beta-thalassemia major patients have an increased susceptibility to infection, which is attributable to a number of immune abnormalities.