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Immune Thrombocytopenic Purpura As Sole Manifestation And Case Report / Olgu Sunumu DOI: 10.5578/ced.57487 • (J Pediatr Inf 2017; 11: i35-i38) Immune Thrombocytopenic Purpura As Sole Manifestation and Hemophagocytic Lypmhohistiocytosis As Sole Manifestation of Hepatitis a Virus Infection in Two Children* İmmün Trombositopenik Purpuranın Tek Bulgu Olduğu ve Hemofagositik Lenfohistiositozun Tek Bulgu Olduğu İki Çocuk Olguda Hepatit A Virüs Enfeksiyonu Zümrüt Şahbudak Bal1, Semra Şen1, Nihal Karadaş Özdemir2, Katibe Başak Yıldız1, Deniz Yılmaz Karapınar2, Fadıl Vardar1 1 Department of Pediatric Infectious Diseases, Ege University School of Medicine, Izmir, Turkey 2 Department of Hematology, Ege University School of Medicine, Izmir, Turkey * This case report was poster presented at ESPID Congress. Abstract Özet Hepatitis A virus (HAV) infection is often asymptomatic or mildly Hepatit A virüs (HAV) enfeksiyonu çocukluk çağında genellikle symptomatic in pediatric population, the severity of the disease asemptomatik ya da hafif seyirlidir, yaş arttıkça hastalığın ciddiyeti artar. increases by age. Immune-mediated extrahepatic extrahepatic Ekstrahepatik immün-aracılı bulgular ve hematolojik komplikasyonlar manifestations and hematologic complications are mainly reported genellikle yetişkinlerde, akut ve kronik hepatit B ve C enfeksiyonlarının in adults with acute and chronic hepatitis B and C. However, they are seyrinde bildirilmiştir. Çocukluk çağında oldukça nadirdir. İngilizce relatively rare in children with HAV infection. There are few available literatürde, çocuklarda hepatit A’nın otoimmün komplikasyonlarını pediatric reports in English literature about autoimmune complications bildiren olgu sunumları oldukça azdır. Bu çalışmada, immün of HAV infection. We reported hematologic manifestations of HAV trombositopenik purpura ve hemofagositik lenfohistiositoz (HLH) infection including hemophagocytic lymphohistiocytosis (HLH) and bulguları ile HAV tanısı alan iki çocuk olgu sunulmuştur, bu nedenle bu immune thrombocytopenic purpura as sole manifestations of HAV hastalıklarda tanı anında sarılık bulgusu olmasa da HAV enfeksiyonu infection therefore in patients with these hematologic disorders, HAV akılda tutulmalıdır. infection should be kept in mind in the differential diagnosis even if they Anahtar Kelimeler: Hematolojik bulgu, hepatit A, çocuk are not jaundiced. Keywords: Hematologic manifestation, hepatitis A, child Correspondence Address / Yazışma Adresi: ©Copyright 2017 by Pediatric Infectious Diseases Society -Available online at Zümrüt Şahbudak Bal www.cocukenfeksiyon.org Ege Üniversitesi Tıp Fakültesi, Çocuk Enfeksiyon Hastalıkları Bilim Dalı, İzmir-Türkiye ©Telif Hakkı 2017 E-mail: [email protected] Çocuk Enfeksiyon Hastalıkları Derneği -Makale metnine www.cocukenfeksiyon.org web sayfasından ulaşılabilir. Şahbudak Bal et al. i36 Hematologic Manifestations of Hepatitis A J Pediatr Inf 2017; 11: i35-i38 Introduction ve results for anti-HAV IgG, hepatitis B and C, Epstein-Barr virüs, cytomegalovirus, toxoplasma, rubella, and parvovirus B19. He Hepatitis A virus (HAV) infection in children is typically an developed anti-HAV IgG, and serum anti-HAV IgM disappeared acute, self-limited disease associated with general, nonspeci- six weeks later. One month later after admission liver functions fic symptoms, such as fever, malaise, anorexia, and vomiting returned to normal levels. As a result of these findings, ITP as the (1). In children younger than 5 years, HAV infection is usual- sole manifestation of acute hepatitis A infection was diagnosed ly inapparent; whereas, most adults present with symptoms, in our patient. with the severity of the disease increasing with the age (1,2). Case 2 A previously healthy 14- month girl referred to our Autoimmune complications such as idiopathic thrombocyto- hospital with prolonged fever, cough, loss of appetite, and penic purpura (ITP), aplastic anemia, and hemophagocytic ly- malaise. Her physical examination revealed pallor, abdominal mphohistiocytosis (HLH) have been rarely reported in children distension. The liver and spleen were both palpable 8 and 10 (3). Hepatitis A is a vaccine-preventable disease but it was not cm below the costal margin. She was not jaundiced and other included in the national vaccination programme of Turkey be- system examinations had normal findings. She had a history of fore November 2012. Herein, we report hematologic manifes- one sibling’s death and there was no consanguinity between tations associated with HAV infection in two pediatric patients her parents. There was no family history of immune or hemato- without jaundice. logic disorders. Peripheral blood counts showed pancytopenia Case Reports ( white blood cell 2.26 x 10³/mL; hemoglobin 7.57 g/dL; hema- A Case 1 A previously healthy eleven year old boy admit- tocrit 21%; platelets 51 x 10³/mL). Coagulation studies revealed ted to our hospital with the complaints of bleeding in the a prothrombin time of 13 seconds, active partial thromboplas- mouth from oral mucosa, bruising all over the body, and pe- tin time of 29.5 seconds, a fibrinogen level of 130 mg/dL, and a techial rash. Past history did not suggest an underlying liver dimer level over 4500 µ/mL. Blood chemistry revealed elevated disease or hematologic disorder. Several purpuric, petechial liver enzyme levels (AST 546 U/L, ALT 587 U/L), C-reactive pro- lesions and ecchymosis were detected on physical examina- tein (13.6 mg/dL), lactate dehydrogenase enzyme (LDH) (2472 tion in his mouth, upper and lower extremities. His liver was mg/dL), alkaline phosphatase (763 mg/dL), triglyceride (506 2 cm palpable, and spleen was not palpable. Physical exami- mg/dL), ferritin (16.600 µg/dL), and normal serum level levels of nation of the other systems had normal findings. Laboratory total and direct bilirubin, total protein and albumin, and blood investigations revealed elevated liver enzyme levels (AST 2512 urine nitrogen. Bone marrow aspiration showed hemophago- U/L, ALT 2248 U/L), with the normal levels of alkaline phospha- cytosis. Hepatosplenomegaly was the main finding on ultra- tase, total and direct bilirubin, gamma glutamyl transferase, sonography. Her laboratory findings revealed pancytopenia, ferritin, total protein, albumin, prothrombin time, activated elevated triglyceride, and ferritin levels, hypofibrinogenemia, partial thromboplastin time, fibrinogen and D- dimer. Comp- and hemophagocytosis in bone marrow aspiration, and these lete blood cell count showed that hemoglobin was 11 g/dL, findings were fulfilled the diagnostic criteria of hemophagocy- white blood cell count 2870/mL with a differential analysis of tic lymphohistiocytosis (HLH). For the etiology, viral serologic 41% neutrophil, 34% lymphocyte, and 18% monocyte, plate- studies were performed and found positive results for anti-HAV let count 10000/mm3, and reticulocyte count of 0.8%. Direct IgM, and negative results for anti- HAV IgG, hepatitis B and C, and indirect Coombs tests, antinuclear antibody, antidsDNA, Epstein-Barr virus, cytomegalovirus, rubella, and Parvovirus anticardiolipin and antiphospholipid IgM and IgG antibodies B19. Absence of the parental consanguinity and a known fa- were negative. Quantitative serum immunoglobulins and mily history made the diagnosis of secondary HLH likely. Due complement (C3 and C4) levels were normal. Examination of to high endemicity in the South eastern region of Turkey and the bone marrow aspiration revealed normocellular marrow also her young age, we performed her genetic analysis and ge- with trilineage hematopoiesis and megakaryocytic hyperp- netic defect was not detected in PRF 1, SNX 11, UNC13D genes lasia with many mature and immature megakaryocytes and for primary (familial) HLH. She was treated with steroid, and IVIG no hemophagocytosis. His bone marrow aspiration showed at a dose of 0.4 mg/kg/day was prescribed for 5 days. Ferritin increased megakaryocytes so we established the diagnosis level increased up to 15.900 µg/dL again, hypofibrinogenemia of ITP and intravenous immunoglobulin (IVIG ) with a 1 g/kg persisted (97 mg/dL), and her physical examination revealed dose was given for two consequence days, on the third day hepatosplenomegaly. Her general condition did not improve platelet counts was 51.400/mm3, and platelet counts returned despite steroid and IVIG therapy so we started cyclosporine. Her to normal values (369.000/ mm3) in seven days. To investigate laboratory abnormalities improved dramatically after the onset th the etiology of ITP, viral serologic tests were performed and of the cyclosporine. She was in good health at the 6 month of found positive results for anti-HAV IgM antibody, and negati- follow up. Şahbudak Bal et al. J Pediatr Inf 2017; 11: i35-i38 Hematologic Manifestations of Hepatitis A i37 on third and fifth days. Our patient did not respond to IVIG Discussion and steroid therapy so the therapy switched to cyclosporine. Hepatitis A infection is usually a self-limited disease con- Her hematological parameters turned to normal values at 12th ferring lifelong immunity (1-3). HAV infection associated with day but triglyceride and ferritin levels which show activation, hematologic complications including severe aplastic anemia, turned to normal levels after cyclosporine therapy onset. virus associated hemophagocytic syndrome (VAHS), postinfe- Although many viral infections such as hepatitis B, par- ctious thrombocytopenic purpura, and immune thrombocy- vovirus and Epstein-Barr virus are associated with extrahe- topenic purpura (4). Immune-mediated extrahepatic
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