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• W2094445608 abstract "Hemophagocytic lymphohistiocytosis (HLH), also called hemophagocytic syndrome (HS), is characterized by phagocyte activation caused by uncontrolled hyperinflammation due to immune disturbance. The malignancy-associated type (malignancy-associated HS, MAHS) is the most common type of reactive HLH in adults. Intravascular B cell lymphoma and T cell/natural killer cell lymphoma are the major background diseases associated with MAHS [1,2]. In patients with MAHS, both proliferation and activation of normal phagocytes in the monocyte-macrophage lineage are generally found. Consequently, activated phagocytes phagocytose blood cells, resulting in the development of cytopenia. MAHS is also found in patients with other hematopoietic tumors including acute myelogenous leukemia (AML), but less frequently than lymphoma-associated HLH. In some patients with AML-associated HLH, leukemia cells instead of activated phagocytes directly phagocytose blood cells, thereby playing an essential role in phagocytosis. Interestingly, most of those patients have specific chromosomal abnormalities involving 8p11 and 16p13 (table 1). Here, we present a case of HLH associated with AML (M5b) with no chromosomal abnormality, in which leukemia cells primarily phagocytosed blood cells. A 64-year-old man was admitted to our hospital with a 3-day history of high fever and oral bleeding. Hematological examination revealed pancytopenia: his white blood cell count was 0.7 × 109/l with 39% monocytoid blasts, hemoglobin 9.6 g/dl and a platelet count of 21 × 109/l. Bone marrow aspiration disclosed hypocellular bone marrow with 77.2% monocytoid blasts (fig. 1a). The blasts were positive for CD13, CD33, CD56, CD15, CD36, CD34 and CD14, but negative for myeloperoxidase on flow cytometric analysis. Cytochemical and immunohistochemical studies revealed that the blasts were positive for KP1/CD68 (fig. 1b), CD31 and α-naphthyl butyrate esterase and negative for peroxidase and CD34. Chromosomal analysis showed no abnormal karyotype. The level of serum lysozyme was high (108.9 μg/ml). Based on these results, we diagnosed this case as AML (M5b) with no chromosomal abnormality. The patient showed high levels of serum ferritin (77,505.8 ng/ml), lactate dehydrogenase (9,008 U/l), soluble IL-2 receptor (4,040 U/ml) and triglycerides (261 mg/dl). Coagulation examination revealed a reduced fibrinogen level (74 mg/ml) along with elevated levels of D-dimer (538.4 μg/ml) and FDP (1,176.0 μg/ml). In addition, computed tomography showed splenomegaly, ascites and cervical lymphadenopathy. Taken together with these results, a diagnosis of HLH was made according to the 2004 diagnostic guidelines for HLH [2]. Importantly, in addition to a few mature histiocytes, 27.6% of the monocytoid blasts directly phagocytosed blood cells in bone marrow (fig. 1c). Treatment with etoposide (100 mg/body weight) was started for HLH. The fever disappeared the next day and the serum ferritin level declined gradually to 2,619.6 ng/ml on day 8. Subsequently, the patient underwent induction chemotherapy consisting of cytarabine and daunorubicin, and he achieved complete remission. At that time, the serum ferritin level remained high (1,104.3 ng/ml), but the HLH-related clinical symptoms and other laboratory abnormalities completely disappeared. Four further courses of chemotherapy were given as postremission therapy, and there has been no recurrence of AML or HLH since.AML-associated HLH is rare. In fact, Ishii et al. [1 ]reported that in only 9 of 132 patients was the MAHS associated with AML. In our case, there was no other possible cause for secondary HLH, e.g. an infectious or autoimmune disease or a drug allergy. Furthermore, the patient had no malignant diseases except for AML. We therefore concluded that the patient had AML-associated HLH.It has been reported that, in some patients with AML-associated HLH, leukemia cells directly phagocytose blood cells in the bone marrow. Interestingly, most patients in this group have specific chromosomal abnormalities involving 8p11 and 16p13 (table 1), though it has still to be clarified how such abnormalities are involved in the phagocytic activation of leukemia cells. Since many of these patients have acute myelomonocytic leukemia (M4) or acute monocytic leukemia (M5), it is possible that genetic alterations due to chromosomal abnormalities increase the phagocytic ability of monocytic leukemia cells. In fact, t(8;16)(p11;p13) and inv(8)(p11;q13) result in the formation of MOZ-containing fusion genes, and it is therefore possible that MOZ is involved in such genetic alterations. Our patient, however, showed no abnormal chromosomes. This is a very rare case. Indeed, only 1 patient with a normal karyotype was previously reported (table 1). It is noteworthy that the leukemia cells in our patient also showed monocyte-macrophage features; it is likely that these cells primarily had a strong phagocytic ability independent of the chromosomal abnormalities. Jekarl et al. [3 ]showed that the expression level of CD56 is associated with the percentage of hemophagocytic leukemia cells in AML patients with t(16;21)(p11;q22). Since the leukemia cells in our case were positive for CD56, the involvement of CD56 expression in the ability to phagocytose is of great interest. Jordan et al. [2 ]reported that patients with MAHS should be initially treated with immunochemotherapy for suppressing hyperinflammation and subsequently undergo therapy specific for a background malignant disease. In addition, it has been shown that Epstein-Barr virus-HLH patients receiving etoposide within 4 weeks after the diagnosis of HLH have a good prognosis [4]. In our case, the initial administration of etoposide resulted in an improvement of HLH-related clinical symptoms, and the patient was treated successfully with subsequent chemotherapy for AML. We have described a case of AML-associated HLH with a normal karyotype, in which leukemia cells phagocytosed blood cells. Although AML-associated HLH is rare, it is potentially fatal. It is therefore important to elucidate the pathophysiology of this disease.We wish to thank Ms. E. Yamakawa for the preparation of the manuscript." @default.
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• W2094445608 title "Leukemia Cells Directly Phagocytose Blood Cells in AML-Associated Hemophagocytic Lymphohistiocytosis: A Case Report and Review of the Literature" @default.
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• W2094445608 doi "https://doi.org/10.1159/000360840" @default.
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