Recurrent epistaxis with coagulation disorders in a boy aged 2 years / 中国当代儿科杂志

A boy, aged 2 years and 5 months, had recurrent epistaxis, and the coagulation function examination showed that activated partial thromboplastin time (APTT) was significantly prolonged. Further laboratory examinations showed that the prolonged APTT was not immediately corrected in the APTT correction test, with positive lupus anticoagulant and low prothrombin activity. The boy was diagnosed with hypoprothrombinemia-lupus anticoagulant syndrome. The condition was improved after treatment with glucocorticoid, immunoglobulin, and vitamin K1. The boy has been followed up for 6 months, and no epistaxis was observed. Prothrombin activity returned to normal, and lupus anticoagulant remained positive. This is a relatively rare disease, and for patients with bleeding symptoms and coagulation disorders, it is recommended to perform the tests such as APTT correction test, lupus anticoagulant testing, and coagulation factor dilution test, which can improve the detection rate of this disease, so as to achieve early diagnosis, provide rational treatment in the early stage, and improve the prognosis.

Lupus Anticoagulant-hypoprothrombinemia Syndrome that did not Progress to Systemic Lupus Erythematosus: A Case Report / 임상소아혈액종양

Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare disorder, and appears mostly in children. The primary concern is its potential development into systemic lupus erythematosus (SLE). A 5-year-old patient was hospitalized with multiple purpuric lesions. A markedly prolonged prothrombin time and activated partial thromboplastin time were observed and were not corrected after mixing with normal plasma. Decreased factor II activity was consistent with LA-HPS. Identifying risk factors that play an important role in the development of SLE in patients with LA-HPS is of importance. Based on the case described here, anti-double stranded (ds) DNA antibody and the Sapporo criteria for antiphospholipid syndrome are related to subsequent SLE development, whereas there is no correlation with the results of the lupus anticoagulant (LA) test. We recommend an early and serial examination of anti-ds DNA antibody and full evaluation of Sapporo criteria for the screening of patients with LA-HPS who may progress to SLE.

Lupus Anticoagulant-hypoprothrombinemia Syndrome that did not Progress to Systemic Lupus Erythematosus: A Case Report / 임상소아혈액종양

Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare disorder, and appears mostly in children. The primary concern is its potential development into systemic lupus erythematosus (SLE). A 5-year-old patient was hospitalized with multiple purpuric lesions. A markedly prolonged prothrombin time and activated partial thromboplastin time were observed and were not corrected after mixing with normal plasma. Decreased factor II activity was consistent with LA-HPS. Identifying risk factors that play an important role in the development of SLE in patients with LA-HPS is of importance. Based on the case described here, anti-double stranded (ds) DNA antibody and the Sapporo criteria for antiphospholipid syndrome are related to subsequent SLE development, whereas there is no correlation with the results of the lupus anticoagulant (LA) test. We recommend an early and serial examination of anti-ds DNA antibody and full evaluation of Sapporo criteria for the screening of patients with LA-HPS who may progress to SLE.

Lupus Anticoagulant-hypoprothrombinemia Syndrome as an Initial Manifestation of Systemic Lupus Erythematosus: A Case Report / 임상소아혈액종양

The lupus anticoagulant-hypoprothrombinemia syndrome, characterized by presence of lupus anticoagulant with acquired factor II deficiency, is a rare disease entity often presented with acute bleeding episodes. A 15-year-old girl was hospitalized with 3 month history of menorrhagia and easy bruising. Prothrombin time (31.3 sec, normal value: 10-13 sec) and activated partial thromboplastin time (72.5 sec, normal value: 27.5-34.7 sec) were markedly prolonged and partially corrected after mixing with normal plasma. Decreased Factor II activity (4%, normal range: 79-131%) or prolonged dilute Russell's viper venom time (89.8 sec, normal value: 25.4-34.3 sec), was consistent with lupus anticoagulant-hypoprothrombinemia syndrome. Antinuclear antibody, anti-double strand-DNA antibodies and anticardiolipin antibodies were also positive. Bleeding diathesis tends to wax and wane while 5 years of treatment with steroid combined with immunosuppressants, however, there was no more active bleeding episodes. Several years after diagnosis, myocarditis, pericarditis, seizure was occurred, fulfilled the diagnostic criteria of systemic lupus erythematosus.

Pulmonary hemorrhage in pediatric lupus anticoagulant hypoprothrombinemia syndrome / 소아과

Lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a very rare disease that is caused by the presence of antifactor II antibodies, is usually counterbalanced by the prothrombotic effect of lupus anticoagulant (LAC). Patients with LAHPS are treated using fresh frozen plasma, steroids, immunosuppressive agents, and immunoglobulins for managing the disease and controlling hemorrhages. Notably, steroids are the important treatment for treating hypoprothrombinemia and controlling the bleeding. However, some patients suffer from severe, life-threatening hemorrhages, when factor II levels remain very low in spite of treatment with steroids. Here, we report a case of LAHPS in a 15-year-old girl who experienced pulmonary hemorrhage with rapid progression. She was referred to our hospital owing to easy bruising and prolonged bleeding. She was diagnosed with LAHPS that presented with pancytopenia, positive antinuclear antibody, proloned prothrombin time, activated partial thromboplastin time, positive LAC antibody, and factor II deficiency. Her treatment included massive blood transfusion, high-dose methylprednisolone, vitamin K, and immunoglobulin. However, she died due to uncontrolled pulmonary hemorrhage.

Lupus Anticoagulant-hypoprothrombinemia Syndrome as an Initial Manifestation of Systemic Lupus Erythematosus: A Case Report / 임상소아혈액종양

The lupus anticoagulant-hypoprothrombinemia syndrome, characterized by presence of lupus anticoagulant with acquired factor II deficiency, is a rare disease entity often presented with acute bleeding episodes. A 15-year-old girl was hospitalized with 3 month history of menorrhagia and easy bruising. Prothrombin time (31.3 sec, normal value: 10-13 sec) and activated partial thromboplastin time (72.5 sec, normal value: 27.5-34.7 sec) were markedly prolonged and partially corrected after mixing with normal plasma. Decreased Factor II activity (4%, normal range: 79-131%) or prolonged dilute Russell's viper venom time (89.8 sec, normal value: 25.4-34.3 sec), was consistent with lupus anticoagulant-hypoprothrombinemia syndrome. Antinuclear antibody, anti-double strand-DNA antibodies and anticardiolipin antibodies were also positive. Bleeding diathesis tends to wax and wane while 5 years of treatment with steroid combined with immunosuppressants, however, there was no more active bleeding episodes. Several years after diagnosis, myocarditis, pericarditis, seizure was occurred, fulfilled the diagnostic criteria of systemic lupus erythematosus.

Endocardite infecciosa por Haemophilus aphrophilus: relato de caso / Infective endocarditis due to Haemophilus aphrophilus: a case report

OBJETIVO: Descrever o caso de uma criança com endocardite infecciosa causada por Haemophilus aphrophilus. DESCRIÇÃO: Menino com febre e calafrios há 20 dias. À internação, apresentava-se febril, descorado e sem sinais de instabilidade hemodinâmica; à ausculta cardíaca, tinha sopro holosistólico em foco mitral. Os exames laboratoriais identificaram anemia (hemoglobina = 9,14 g/dL), leucócitos totais de 11.920 mm³, plaquetas de 250.000 mm³, velocidade de sedimentação das hemácias e proteína C reativa elevadas. O ecocardiograma revelou imagem em válvula mitral, sugestiva de vegetação. Com a hipótese de endocardite, foi iniciada antibioticoterapia com penicilina cristalina (200.000 UI/kg/dia) associada à gentamicina (4 mg/kg/dia). No terceiro dia de tratamento, foi identificado Haemophilus aphrophilus em hemoculturas, sendo então trocado o esquema antibiótico para ceftriaxona (100 mg/kg/dia). No 20º dia de internação, encontrava-se pálido, mas sem febre e sem outras queixas. Os exames mostravam hemoglobina = 7,0 g/dL, leucócitos = 2.190 mm³, plaquetas = 98.000 mm³, razão normatizada internacional = 1,95 e R = 1,89. Foi feita hipótese de reação adversa ao ceftriaxona, que foi substituído por ciprofloxacina, 20 mg/kg/dia, até completar 6 semanas de tratamento. Após 72 horas da troca, houve normalização dos exames. Durante seguimento ambulatorial, apresentou insuficiência mitral grave, sendo submetido a troca de válvula por prótese metálica 9 meses após quadro agudo. Há 3 anos encontra-se bem, em acompanhamento ambulatorial. COMENTÁRIOS: É rara a identificação de agentes do grupo HACEK (Haemophilus ssp, Actinobacillus actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens e Kingella kingae) em crianças com endocardite infecciosa. O caso apresentado, sem fatores de risco relacionados a esses agentes, reafirma a necessidade de tentar sempre identificar o agente etiológico das endocardites para adequação do tratamento.

OBJECTIVE: To report the case of a child with infective endocarditis caused by Haemophilus aphrophilus. DESCRIPTION: Boy with 20 days of fever and chills. On admission, he was febrile, pale and with no signs of hemodynamic instability; on cardiac auscultation, a mitral-related holosystolic murmur was observed. Laboratory examination identified anemia (hemoglobin = 9.14 g/dL), total leukocytes of 11,920 mm³, platelets of 250,000 mm³, elevated sedimentation velocity of red cells and elevated C-reactive protein. The echocardiogram revealed image on mitral valve, resembling vegetation. Considering endocarditis, antibiotic therapy was started with crystalline penicillin (200,000 UI/kg/day) in association with gentamicin (4 mg/kg/day). On the third day of treatment, Haemophilus aphrophilus was identified in the blood cultures and the antibiotic scheme was replaced with ceftriaxone (100 mg/kg/day). On the 20th day of evolution, the patient was pale but with no fever or other complaints. Examinations showed hemoglobin = 7.0 g/dL, leukocytes = 2,190 mm³, platelets = 98,000 mm³, international normalized ratio = 1.95 and R = 1.89. Considering the hypothesis of adverse reaction to ceftriaxone, a 6-week replacement treatment with ciprofloxacin (20 mg/kg/day) was started. Examination results normalized after 72 hours of the replacement therapy. During ambulatory follow-up, patient presented with severe mitral regurgitation, undergoing a valve replacement with a metallic prosthetic valve 9 months after acute event. Patient has done well throughout the 3-year ambulatory follow-up. COMMENTS: Identification of agents of the HACEK group (Haemophilus ssp, Actinobacillus actinomycetemcomitans,Cardiobacterium hominis, Eikenella corrodens and Kingella kingae) in children with infective endocarditis is rare. This case report, with no HACEK agent-related risk factors, reinforces the need for identification of the etiological agent of endocarditis to ensure adequate treatment.

Lupus anticoagulant-hypoprothrombinemia in healthy adult

The presence of lupus anticoagulant is associated with an elevated risk of venous and arterial thrombosis, and recurrent miscarriages as well. For some cases, this disease can present with bleeding as a consequence of lupus anticoagulant hypoprothrombinemia (LAHPS). LAHPS is a rare disease and it is reported to be most frequent in young females with/without systemic lupus erythematosus or in healthy children who are suffering with a viral infection. In such cases, steroid therapy is usually effective in normalizing the biological abnormalities and controlling the bleeding problems. A 34-year-old previously healthy man was admitted to our department because of his prolonged coagulation times; these abnormalities were discovered before performing orthopedic surgery. The prothrombin time (PT) was 15.2 sec, and the activated partial thromboplastin time (APTT) was 37.7 sec. A 1:1 dilution of patient plasma with normal plasma nearly corrected the PT, but this failed to correct the APTT. Evaluation of the clotting factors revealed decreased levels of factors II, V, VIII, IX and XI. The presence of LA was demonstrated by the dRVVT test, and the patient was diagnosed with LAHPS. He was successfully treated with corticosteroid before performing the orthopedic surgery.