CONTEXT Hemophagocytic syndrome (HPS) is mainly characterized by massive infiltration of bone marrow by activated macrophages and often presents with pancytopenia. Thrombotic microangiopathy (TMA) is also present with thrombocytopenia and renal involvement. Both conditions could coexist with each other and complicate the condition. EVIDENCE ACQUISITION Directory of Open Access Journals (DOAJ)...

A 17-year-old male presented with progressive splenomegaly since age 7 years, recurrent epistaxis and skin bleeds for the last 5 years, and worsening dyspnoea (initially on exertion progressing to symptoms at rest) for the last 3 years. Examination showed evidence of hypoxemia (blood oxygen saturation on room air, 70%; on 3 L/min oxygen, 92%, with orthodeoxia), clubbing, peripheral cyanosis, pe...

Hyperplastic polyposis syndrome (HPS) is a rare condition characterized by the presence of numerous hyperplastic polyps (HPs) in the colon and rectum. Patients with HPS have an increased risk of colorectal cancer. This link is associated with gene mutations, especially B type Raf kinase (BRAF). However, a case of HPS associated with gene mutations has seldom been reported in Korea. Here, we des...

Reactive hemophagocytic syndrome (HPS) is characterized by hemophagocytosis by activated histiocytes, resulting in pancytopenia and liver dysfunction. We describe a patient with adult onset Still's disease (AOSD) in whom HPS developed. An 80-year-old Japanese woman with high fever, arthralgia, skin rash, and pleuritis was admitted to our hospital for further examination. She was diagnosed with ...