[The role of Helicobacter pylori infection in hematological diseases - a review].

Helicobacter pylori (H. pylori) infection has been known to be the most closely associated with extra-gastrointestinal diseases. Above all, the association between H. pylori and hematological diseases, including immune thrombocytopenic purpura ( ITP), gastric MALT lymphoma and iron deficiency anemia (IDA) has been focused. Although the molecular mechanisms have not yet been fully understood, H. pylori eradication resulted in high response rates without major adverse effects. We focus here on a comprehensive review of the current literature of ITP, gastric MALT lymphoma and IDA.

Pathogenesis, laboratory, and clinical characteristics of Helicobacter pylori-associated immune thrombocytopenic purpura.

Idiopathic thrombocytopenic purpura (ITP) is a common autoimmune disease mediated by autoantibodies against platelet glycoproteins. This hemorrhagic disorder may be primary or secondary to various illnesses, including lymphoproliferative, autoimmune, or infectious diseases. Among the latter causes, there is increasing laboratory and clinical evidence that documents a pathogenic role of Helicobacter pylori infection in ITP. The aim of this review is to analyze the current knowledge on the pathogenic, diagnostic, clinical, and therapeutic characteristics of H. pylori-associated ITP.

[Hemostatic disorders and their implication in the pathogenesis of Astrakhan rickettsial fever].

AIM: to define a role of hemostatic disorders in the pathogenesis of Astrakhan rickettsial fever (ARL). SUBJECTS AND METHODS: Platelet functional activity and plasma hemostatic parameters were studied in 89 patients of moderate ARL. RESULTS: The clinical manifestations of hemostatic disorders at the height of ARL were characterized by the appearance of typhoid maculopapular rashes in 91.4% of the patients, solitary elements of which were transformed to petechiae in 20% of cases. At convalescence (on day 10.2 +/- 1.3 of the disease), all eruptions regressed via pigmentation. At the peak of the fever, there were nasal hemorrhages and bleedings from the sites of injections; lowered platelet aggregation was detectable in the presence of thrombocytopenia at the height. Coagulation hemostasis changes were characterized only by elevated fibrinogen levels. Increased platelet functional activity and decreased fibrinogen concentrations were observed at convalescence. CONCLUSION: The basis of the clinical manifestations of ARL is hemostatic disorders due to thrombocytopenia and diminished platelet functional activity. In early convalescence, there was improved platelet aggregatability; however, the increasing trend for the rate of aggregation and the radius of aggregates suggests a risk of thrombogenesis in convalescents, which requires their follow-up with obligatory hemostatic monitoring.

Clinical surveillance of thrombotic microangiopathies in Scotland, 2003-2005.

The prevalence, incidence and outcomes of haemolytic uraemic syndrome (HUS) and thrombotic thrombocytopaenic purpura (TTP) are not well established in adults or children from prospective studies. We sought to identify both outcomes and current management strategies using prospective, national surveillance of HUS and TTP, from 2003 to 2005 inclusive. We also investigated the links between these disorders and factors implicated in the aetiology of HUS and TTP including infections, chemotherapy, and immunosuppression. Most cases of HUS were caused by verocytotoxin-producing Escherichia coli (VTEC), of which serotype O157 predominated, although other serotypes were identified. The list of predisposing factors for TTP was more varied although use of immunosuppressive agents and severe sepsis, were the most frequent precipitants. The study demonstrates that while differentiating between HUS and TTP is sometimes difficult, in most cases the two syndromes have quite different predisposing factors and clinical parameters, enabling clinical and epidemiological profiling for these disorders.

Thrombocytopenic purpura as only manifestation of brucellosis in a child.

Thrombocytopenic purpura associated with brucellosis has been rarely reported in the world literature. Thrombocytopenic purpura is generally part of the array of manifestations of brucellosis such as fever, arthritis, malaise and hepatosplenomegaly. We describe a nine-year-old girl who presented with thrombocytopenic purpura as the sole manifestation of brucellosis, which resolved with anti-Brucella chemotherapy. Her physical examination was remarkable for hepatomegaly of 3 cm and splenomegaly of 2 cm palpable below the costal margin. Initial laboratory investigations revealed isolated thrombocytopenia with platelet count of 11,300/mm3 and positive serology for Brucella. Thrombocytopenia resolved promptly with proper antibiotics on 7th day of treatment. Brucellosis should be included in the differential diagnosis of thrombocytopenic purpura in Brucella-endemic areas.

Thrombocytopenic purpura associated with miliary tuberculosis.

The authors report a case of thrombocytopenia associated with miliary tuberculosis. The patient was a 28-year-old woman who was admitted because of massive upper gastrointestinal hemorrhage and acute respiratory failure. Chest radiographs revealed diffuse bilateral reticulonodular infiltration and complete blood count was significant for severe thrombocytopenia. Bone marrow biopsy was performed to investigate the cause of thrombocytopenia and demonstrated multiple tiny caseating granulomas suggesting miliary tuberculosis (TB). She received anti-TB therapy and a short course of steroid with good response. Platelet count returned to normal limit within 10 days. Although isolated thrombocytopenia is uncommon in TB, it is still important to consider TB in the differential diagnosis of thrombocytopenia, particularly in patients with abnormal chest radiographs. Bone marrow examination is very helpful in this situation.

Immune thrombocytopenic purpura associated with Brucella and Toxoplasma infections.

Bacterial and protozoal infections can cause thrombocytopenia and may mimic idiopathic thrombocytopenic purpura (ITP). Brucella species and Toxoplasma are among the infectious agents with protean clinical manifestations which may induce immune thrombocytopenia. In rare cases, thrombocytopenia can be severe and may result bleeding into the skin and from mucosal sites. Prompt recognition of this complication and aggressive therapy are essential, since the mortality associated with bleeding into the central nervous system is high. We report two patients with complaints of severe epistaxis and thrombocytopenia associated with brucellosis and toxoplasmosis. Thrombocytopenic purpura in these cases responded well to the high-dose corticosteroid treatment with platelet recovery within 2-3 days. For cases with infection-induced immune thrombocytopenic purpura, short-term high-dose corticosteroids may be applied as an urgent therapy without worsening of the clinical condition.

[Non-characteristic course of disease after dog bite]. / Ukarakteristisk sygdomsforløb efter hundebid.

The case presents a healthy woman in her fifties, who was admitted to hospital with clinical signs of diffuse or disseminated intravascular coagulation and thrombotic thrombocytopenic purpura even though she was respiratorily and circulatorily stable. She developed gastroenteritis four days after a dog bite. After a while she got painful and ischemic legs, and this was the cause of admission. The laboratory manifestations included severe thrombocytopenia, fibrin degradation products but no presence of schistocytes or fragmented red blood cells. A gramnegative rod was seen in the blood but no specific culture was grown. The patient recovered on antibiotic regime within few weeks.

[Capnocytophaga canimorsus bacteremia]. / Capnocytophaga canimorsus-bakteriaemi.

A case of bacteraemia in a 54-year-old previously healthy man caused by the gram-negative rod Capnocytophaga canimorsus is presented. The patient developed severe ischaemia in both feet, thrombocytopenic purpura and renal failure. The bacteraemia was apparently caused by saliva from the patient's dog who had licked ulcers on the patient's legs. The patient regained full health but had a minor inferior myocardial infarction seven weeks after start of the infection.

[Thrombocytopenia in cytomegalovirus infection]. / Trombocytopeni ved cytomegalovirus-infektion.

A case of a 15 month old female patient with thrombocytopenic purpura induced by postnatally acquired Cytomegalovirus (CMV) infection is described. Treatment with intravenous immunoglobulin caused a satisfactory but short-lived attainment of normal platelet count. Spontaneous recovery took place after ten months. A review of the literature up to 1993 revealed that medical treatment of severe thrombocytopenia in normal hosts with the same aetiology had been reported in only five cases, Prednisone treatment was given in four cases (all adults), with no apparent effect on platelet count. Persistent severe thrombocytopenia in a one-year-old child responded to repeated intravenous infusions of immunoglobulin.

[24 cases of human parvovirus B19 infection in children]. / A propos de vingt-quatre observations d'infections à parvovirus humain B19 chez l'enfant.

From January 1, 1987 through December 31, 1990, twenty-four pediatric patients with human parvovirus B19 (HPV B19) infection were seen. In every case the diagnosis was established by a positive capture immunoassay for IgM antibodies against the HPV B19. Four patients had hematologic manifestations, including one case of transient bone marrow aplasia revealing hereditary spherocytosis, one case of autoimmune hemolytic anemia with beta-thalassemia, and two cases of peripheral thrombocytopenia. Eight patients had skin lesions, with a morbilliform rash in six cases, erythema nodosum in one case, and Gianotti-Crosti syndrome in one case. No patients had erythema infectiosum. Seven patients developed joint manifestations: Henoch-Schönlein purpura in two cases, arthralgia in four cases, and polyarticular disease progressing to severe rheumatoid arthritis in a thirteen-year-old girl. Unremarkable symptoms of viral disease were seen in three patients. A five-month-old infant developed severe acute myocarditis. One patient with hepatitis A had acute liver failure. This study confirms the broad spectrum of clinical manifestations of HPV B19 infection. There were a number of unusual findings, including the high rate of joint manifestations (29%) and the severe course of some hematologic and myocardial manifestations. These results raise the question of whether the HPV B19 may be involved in the genesis of chronic juvenile arthritis.

Inhibition of platelet GPIIb/IIIa binding to fibrinogen by serum factors: studies of circulating immune complexes and platelet antibodies in patients with hemophilia, immune thrombocytopenic purpura, human immunodeficiency virus-related immune thrombocytopenic purpura, and systemic lupus erythematosus.

We have studied the conditions of in vitro binding of platelet glycoprotein IIb/IIIa (GPIIb/IIIa) to fibrinogen and applied the results to identify and measure the serum inhibitors to the binding. For the enzyme-linked immunosorbent assay, platelet extract was delivered to a fibrinogen-coated microtiter plate that was incubated for 2 hours, followed by incubation with anti-GPIIb/IIIa monoclonal antibody for another 2 hours. The plate was then incubated with peroxidase-conjugated anti-mouse IgG for color development. The binding was shown to be calcium-dependent. The binding was partially blocked by treating the coated fibrinogen with anti-fibrinogen antibody. Reduction or dissociation of GPIIb/IIIa resulted in the total loss of its ability to bind to fibrinogen. Platelet extracts of patients with hemophilia showed decreased binding (25% and 14%, compared with control platelet extract), and an extract from a patient with Glanzmann's thrombasthenia showed no binding. With the enzyme-linked immunosorbent assay we have measured serum inhibitors to GPIIb/IIIa binding to fibrinogen in 35 hemophilia A, 17 immune thrombocytopenic purpura, 22 human immunodeficiency virus-related immune thrombocytopenic purpura, and 29 systemic lupus erythematosus serum samples. In those patients with inhibition by serum, polyethylene glycol precipitation of circulating immune complexes (CICs) decreased the inhibition by the supernatants, and all the resolubilized CIC precipitates demonstrated inhibition, which indicates that CICs play a major role in the inhibition of GPIIb/IIIa binding to fibrinogen. This, then, provides evidence of CIC-mediated impaired GPIIb/IIIa binding to fibrinogen in hemophilia A, HIV-ITP, and SLE.

[Thrombocytopenic purpura of the young infant caused by perinatal infection by cytomegaloviruses]. / Púrpura trombocitopénica del lactante menor causada por la infección perinatal por citomegalovirus.

Two cases of idiopathic trombocytopenic purpura, in infants 37 and 42 days old, and one case of atypical congenital cytomegaloviral disease are presented, the latter proved at necropsy. Cytomegalic inclusion cells are found in urine in both cases of purpura with hepatosplenomegaly and mononucleosis syndrome. In one of the cases, cytomegalovirus cultures are positive in urine and blood. The hemorrhagic syndrome is mild and recuperation is achieved in less than thirty days, without relapse. The use of corticosteroids is not recommended. With these two new cases, thirteen similar clinical observation of thrombocytopenic purpura in infants, six of them in 1977, are completed. Thus, a new entity is added to the growing list of clinical manifestations caused by human CMV infection.

L- and conventional forms of micrococci in the circulating blood of thrombocytopenic patients.

The multiplication of Gram-positive Cocci originating from L-forms carried by platelets of autoimmune thrombocytopenic patients, may be attributed to the primary platelet damage enhanced following interaction with bacteria.