Hereditary spherocytosis

Hereditary spherocytosis (HS) is a relatively common hemolytic anemia; most affected individuals have mild or only moderate haemolysis. It is a heterogeneous group of disorders with regard to clinical severity, protein defects and mode of inheritance. There is usually a family history, and a typical clinical and laboratory picture so that the diagnosis is often easily made without additional laboratory tests. Atypical cases may require measurement of erythrocyte membrane proteins to clarify the nature of the membrane disorder and in the absence of a family history. Mild HS can be managed without folate supplementation and does not require splenectomy. Moderately or severely affected individuals are likely to benefit from splenectomy, which should be performed after the age of 5-6 years. (Turk Arch Ped 2009; 44 Suppl: 27-34

Aplastic anemia presenting as hemophagocytic lymphohistiocytosis

Two unusual cases of hemophagocytic lymphohistiocytosis (HLH) complicating aplastic anemia (AA) are described. Each patient had a history of preexisting acute hepatitis of unknown cause at the time of HLH diagnosis and infection-associated secondary HLH. They developed high fever and pancytopenia. Hemophagocytes were seen in the bone marrow. With steroid (in combination with etoposide and CyA in 1 patient), high fever disappeared and the patients' liver function gradually recovered. As severe pancytopenia persisted, bone marrow became acellular and AA was diagnosed. Since HLH is known to be able to cause an aplastic bone marrow if untreated for a prolonged time, it is therefore in line that hepatitis-associated AA may also be associated with HLH

2011 yılında çocukluk çağı immün trombositopenik purpura hastalığı izlem ve tedavisinde değişiklikler

Immune thrombocytopenic purpura (ITP) is the most frequent hemorrhagic disease in children. The shortened life of platelets because of immunologic damage (antibodies absorbed by platelets) and insufficient compensatory increased function of the bone marrow result with reduced number of platelets in the peripheral blood. There are three forms of ITP: acute, chronic and persistent. The acute form occurs in 80-90% of cases with bleeding episodes lasting a few days or weeks, but no longer than 6 months, the new 2011 American Society of Hematology guideline increased this period to one year. It is typical for the phenomenon of bleeding that it starts suddenly and without any other sign of illness. The most frequent acute form appears between the second and fourth year, and is occurrence usually after acute viral infections. Children older than 10 years of age, like adults, often have the chronic form associated with other immunologic disorders. Hemorrhagic manifestations include: petechiae, purpura, epistaxis, gastrointestinal and genitourinary bleeding. They depend on the grade of thrombocytopenia, although there is no strict correlation between the number of platelets and volume of bleeding. In cases of acute ITP in children, usually there are two therapeutic options or therapies of choice: corticosteroids and high doses of intravenous immunoglobulin. There are also immunosupressive therapy, anti Rh(D) immunoglobulin, cyclosporine, cytostatics, danazol, rituximab, and thrombopoietin receptor agonists. In cases of distinctive hemorrhagic syndrome there are also indications for platelet transfusion. Nowadays splenectomy is more restricted, because one third of cases is unsuccessful. (Turk Arch Ped 2012; 47:8-16

What does a hemogram say to us?

The most commonly performed blood test is complete blood cell count. This test includes hemoglobin, white blood cell count, platelet count, and detailed red blood cell indices. Automated complete blood count also give information for "differential" which gives information about percentages and absolute numbers of different subgroups of white blood cells. This test is necessary in diagnosing anemia, hematological cancers, infections, acute hemorrhagic states, allergies, and immunod-eficiencies. Also it is used for monitoring side effects of certain drugs. A pediatrician is frequently challenged for evaluating complete blood count as a part patient's assessment. An enhanced and complete understanding of this laboratory test is essential for providing quality care of sick and normal children. Here in this paper, we want to share key laboratory interpretation strategies for complete blood count and some clues for differentiating normal from deviations and true problems

Çocukluk Çağı Lösemilerinin Geç Komplikasyonları

Çocukluk çağı lösemilerinde sağ kalımın %70-90’lara ulaşması tedaviye bağlı geç yan etkilere daha fazla dikkat çekmeye başlamıştır. Lösemi tedavisinin en önemli komplikasyonlarından biri tedavilere ikincil gelişen malignitelerdir. İkincil maligniteler lösemi nüksünden sonra, bu hastalarda ölümün en sık nedenini oluşturur. Lösemi sonrasında ikincil malignite riski izlem süresi attıkça artmaktadır. Akut lenfoblastik lösemi (ALL) sonrasında beyin tümörleri, hematolojik maligniteler (akut miyeloid lösemi AML, miyelodisplastik sendrom MDS), tiroid karsinomu melanom, meme kanseri Hodgkin-dışı lenfoma, parotis karsinomu ve daha nadir tüm.rler gelişebilir. Lösemi tedavisinde kemik iliği transplantasyonu (KİT) uygulanması, ikincil malignite gelişme riskini arttırır. Çocukluk çağında lösemi tedavisi alan hastaların gelişebilecek ikincil malignitelerin erken tanısı ve kullanılan tedavilerin geç dönem yan etkileri açısından yaşam boyu dikkatli bir klinik değerlendirmeyle izlenmeleri gereklidir

Spurious thrombocytopenia

Nowadays automated blood cell analysis is being widely used. When the history and physical examination do not suggest a defect in hemostasis, the thrombocyte count is found low in blood with the anticogulants EDTA or citrate although a blood film prepared directly from blood optained by finger puncture reveales normal thrombocytes. This thrombocytopenia is called pseudo or spurious thrombocytopenia. Here, sharing our experience on a pseudothromboctopenic child, we want to point to this topic altough it is less common in pediatric patients than adults. (Turk Arch Ped 2009; 44: 73-4

Control of bleeding associated with hemophagocytic syndrome in children: An audit of the clinical use of recombinant activated factor VII

This paper presents 2 cases of hemophagocytic lymphohistiocytosis (HLH) in whom recombinant factor VIIa ( rFVIIa) was used for the management of hemorrhage. Both patients were diagnosed as HLH and were bleeding from the gut, which could not be controlled. Patients received rFVIIa at total doses of between 90 and 180 mu g/kg body weight. Hemostatic affect was achieved in both of the patients but lasted only a short time. The response was achieved after 1 h of administration of rFVIIa, lasting for 24 h. The use of rFVIIa was well tolerated. These 2 patients suggest that rFVIIa is a beneficial agent in the management of hemorrhage in patients with HLH, although for a permanent homeostasis the control of primary disease is essential