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Histiocytosis and Lipid Storage Diseases
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Salwa Shabbir Sheikh, David F. Garvin
The common denominator in all these clinical conditions with hemophagocytosis is an injury to the lymphocytes with alterations in recognition of certain antigens, including those on red blood cells with expansion of the antigen-presenting cells or histiocytes which lead eventually to hemophagocytosis.
Hemophagocytic lymphohistiocytosis
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
Aradhana Sood, Deep Kumar Raman, Pankaj Das
Bone marrow studies are mandatory in suspected HLH cases to detect hemophagocytosis and to rule out other causes of cytopenias and malignancies. The cellularity of the marrow is variable, and hemophagocytosis is seen in 25%–100% of HLH but may not be evident in the initial stage of the disease [20]. Macrophages can be highlighted by CD163 staining.
SARS-CoV Infections in Humans
Published in Sunit K. Singh, Human Respiratory Viral Infections, 2014
Some of the clinical features of SARS suggested that exaggerated immune responses might be responsible for tissue damage. A fall in viral load when IgG sero-conversion occurred could be associated with clinical deterioration. The BOOP pattern was observed in the radiological imaging of some patients and confirmed in autopsies in some lethal cases. Hemophagocytosis had been also observed in the lung in autopsy series.
Etoposide-containing regimens for the treatment of critically ill patients with hematological malignancy-related hemophagocytic lymphohistiocytosis
Published in Acta Oncologica, 2022
Clara Vigneron, Valentine Le Stang, Justine Decroocq, Edwige Péju, Barbara Burroni, Nicolas Chapuis, Julien Charpentier, Frédéric Pène
Twenty-four patients were included in the study (Table 1). Sixteen patients fulfilled at least five HLH-2004 diagnostic criteria. The H-score was computed at high values of 253 [226.3–287.0], corresponding to HLH probability higher than 99% [98–>99]. Eight patients had less than five HLH-2004 criteria but with a median H score of 226 [178.3–243.5] resulting in a HLH probability of 98%. Figures of hemophagocytosis were found in 13 patients (56.5%). Hematological malignancies were generally related to mature T and B lymphoma (n = 17, 70.8%), and were often recently diagnosed (n = 20, 83.3%). Supports for organ failures were frequently initiated, including invasive mechanical ventilation (n = 13, 54.2%), vasopressors (n = 17, 70.8%), or renal replacement therapy (n = 13, 54.2%). Etoposide (median dose of 97.7 mg/m2 [86.5–105.5] per day for one or two injections) was administered early (median 0.5 d [0–2.25]) after the diagnosis of HLH was set, and was combined with high-dose steroids in 21 patients (87.5%). Four patients died rapidly before receiving additional chemotherapy. Disease-directed chemotherapy was initiated in 16 patients (58.3%) within one month following ICU admission (median of 4 d [1.5–7.0] from etoposide) and was initiated after one month in four patients.
Clinical Characteristics and Outcomes of 101 Children with Hemophagocytic Lymphohistiocytosis: A Four-Year Single-Center Experience from Egypt
Published in Pediatric Hematology and Oncology, 2020
Asmaa Elsharkawy, Hala Assem, Mostafa Salama, Neveen Mikhael, Maha Y. Zeid, Yasmine El Chazli
Bicytopenia was reported in only 83% of our patients studied, as compared to 92% of the patients reported by Bergsten et al21 and 100% in many studies,6,22,30 this may be explained by the presence of pHLH not presenting with a full-blown picture in the present study. Tissue hemophagocytosis, which is not essential for the diagnosis of HLH,14 was observed in 33 patients, corresponding to 43% of those biopsied, almost exclusively from the BM. Gupta et al31 and Sasan et al32 observed hemophagocytosis in 57% and less than 20% of their patients, respectively. In contrast, the results of the HLH-2004 study showed hemophagocytosis in 82% of the available examinations.21 The considerable variation seen in different studies might be explained by the different timing and procedure for sampling. In our center, patients had a BM examination (aspirate and/or biopsy) only at diagnosis and it was not repeated if negative.
Macrophage activation syndrome in a patient with systemic lupus erythematosus (SLE) and dual viremia
Published in Journal of Community Hospital Internal Medicine Perspectives, 2020
Shankar Awasthi, Sunita Upreti
Macrophage activation syndrome (MAS) has been commonly reported in pediatric population as a complication sJIA, but cases in adults have emerged with the association of MAS with a variety of inflammatory conditions, like malignancy, infection (i.e., Epstein-Barr virus), and primary immunodeficiencies [1]. Hemophagocytosis is defined as the engulfment of blood cells, including red blood cells, white blood cells, or platelets by phagocytic cells. This can be detected by serum laboratory tests like soluble interleukin 2 receptor alpha chain (sCD25) and soluble CD163 (sCD163), a high-affinity scavenger receptor for hemoglobin-haptoglobin complexes [1]. This clinical syndrome was first described in the 1980s in children with severe cases of sJIA [1]. Due to rapid development of multiorgan failure most patients are admitted to the Intensive Care unit (ICU). The frequency of ICU admission from MAS has been reported as 43.7%, with the mortality rate as high as 11.4% [2]. Clinical and laboratory features of MAS include sustained fever, hyperferritinemia, pancytopenia, fibrinolytic coagulopathy, and liver dysfunction [1,3].