**7. References**


In summary, the diagnosis and management of chronic ITP require a highly individualized approach that is often based on the natural history of the disease in the particular patient as well as the experience of the physician. Published guidelines can certainly provide some guidance to the physicians but the decision of when and how to treat the thrombocytopenia will still depend upon the hematologists. Although ITP is an old disease, our recent understanding of the pathogenesis has caused a marked paradigm shift. For decades, steroids and IVIg have remained frontline treatment. Recently, the developments of newer therapies, such as rituximab and thrombopoietic agonists, have had a major impact on the management of ITP. A combination of different agents may be a useful approach in the future but a single uniform set of guidelines is difficult to establish. To date, optimizing the

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**6. Conclusion** 

**7. References** 

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**5** 

*1Sweden 2,3 Poland* 

**Autoimmune-Associated Hemophagocytic** 

Maciej Machaczka1, Wojciech Sydor2, Małgorzata Rucińska3,

*1Hematology Center, Karolinska University Hospital Huddinge, Stockholm* 

Marta Szostek3 and Jacek Musiał<sup>2</sup>

*Jagiellonian University, Kraków* 

*2The Second Chair and Department of Internal Medicine, Collegium Medicum,Jagiellonian University, Kraków* 

*3Chair and Department of Hematology, Collegium Medicum,* 

**Syndrome/Macrophage Activation Syndrome** 

Hemophagocytic syndrome (HPS), also known as hemophagocytic lymphohistiocytosis (HLH) encompasses an infrequent group of non-malignant, yet potentially life-threatening disorders caused by massive cytokine release from activated lymphocytes and macrophages (Filipovich, 2009; Henter et al., 1998, 2007; Janka et al., 1998; Janka, 2009). This multisystem inflammatory syndrome is associated with a range of genetic and acquired factors. Hectic and persistent fever, cytopenias, hepatitis, jaundice, edema, splenomegaly, neurological symptoms and hemophagocytosis in bone marrow (BM), liver or lymph nodes are common

The first published report on HLH is presumably an observation of hemophagocytosis in malignancy (Tschistowitsch & Bykova, 1928). In 1939, Scott and Robb-Smith reported four similar cases of adults with an HLH-like picture and proposed to call this condition histiocytic medullary reticulosis (HMR) (Scott & Robb-Smith, 1939). The term HMR was later succeeded by the disease entity known as malignant histiocytosis (MH) (Rappaport, 1966). The familial form of HLH (named FHL or FHLH) was first described in a family with two affected siblings (Farquhar & Claireaux, 1952). Risdall et al. later reported a series of 19 patients with active viral infection, whose bone marrow smears disclosed histiocytic hyperplasia with prominent hemophagocytosis (Risdall et al., 1979). Of note, 14 of 19 patients in this study were immunosuppressed and active infection with herpes group viruses was documented in 74% (14/19) of patients. They proposed to term this condition virus-associated hemophagocytic syndrome (VAHS). Some authors argue that this paper by Risdall and colleagues is the first well documented report of acquired (or secondary) HLH (sHLH) (Kumakura, 2005). Five years later, Risdall et al. also reported HPS in three patients with bacterial sepsis (Risdall et al., 1984). This condition was named bacteria-associated

**1. Introduction** 

clinicopathological features of HLH.

**2. Historical background and terminology of HLH** 

