Thrombosis and haemostasis

Colman, Robert W.; Stewart, G. J.; Budzyński, A. Z.; Ittyerah, T. Roy; Olexa, Stephanie A.; Kirby, Edward P.; Fukami, Miriam H.; Piperno, Jeanette R.

doi:10.1038/282676a0

Cited by 5 publications

(5 citation statements)

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“…FFP therapy and/or APC therapy was administered to all the studied children. Notably, PC replacement to 4%-10% of normal adult levels is reportedly adequate to suppress thrombin generation in PC-deficient children and to prevent PF [12,17,19].…”

Section: Discussionmentioning

confidence: 98%

“…Any skin necrosis in a child with PC deficiency requires replacement therapy with fresh-frozen plasma (FFP) or PC concentrate (along with anticoagulants) in order to suppress the intense activation of coagulation [12,16]. However, PC zymogene concentrate is not always available for use in acute PF; thus, APC concentrate, a physiologic anticoagulant, reported as being a mode for decreasing mortality in adults with sepsis [17], has also been attempted in children with PF [18].…”

Section: Introductionmentioning

confidence: 99%

“…Both severe PC and PS deficiencies are characterized by episodes of PF [10,11] and require prolonged anticoagulant therapy. Even with intensive anticoagulation, however, affected children may be susceptible to periodic recurrent bouts of PF [12][13][14][15].…”

Section: Introductionmentioning

confidence: 99%

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Skin Necrosis and Purpura Fulminans in Children With and Without Thrombophilia—A Tertiary Center's Experience

Fruchtman

Strauss

Rubinstein

et al. 2015

Pediatric Hematology and Oncology

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Purpura fulminans (PF) is a very rare clinicopathologic skin disorder comprising dermal microvascular thrombosis associated with perivascular hemorrhage of multiple origins. It may occur as the presenting symptom of severe congenital deficiency of protein C (PC) or protein S (PS) during the newborn period, or later in life following oral anticoagulant therapy with vitamin K antagonists, or of sepsis that may be associated with disseminated intravascular coagulation. Treatment consists of anticoagulants and PC concentrates during acute episodes. We report our experience in the diagnosis and management of pediatric PF. The medical records of the 6 children aged 2-16 years (median: 5 years) who presented with PF to our tertiary care center between 1996 and 2013 were studied. The thrombophilia workup revealed either the presence of congenital homozygous PC deficiency, prothrombotic polymorphisms (factor V Leiden and FIIG20210A heterozygosity), acquired PC/PS deficiency, or no discernible thrombophilia. The skin necrosis resolved following conservative fresh-frozen plasma/anticoagulant therapy in 2 cases, whereas 3 children required interventional plastic surgery. The sixth case, a 10-year-old child with severe PC deficiency, heterozygous factor V Leiden, and FIIG20210A, received recombinant activated PC. PF in childhood is rare and has multiple etiologies. Understanding of the variable pathogenesis and risk factors will facilitate diagnosis and appropriate clinical management.

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Section: Discussionmentioning

confidence: 98%

Section: Introductionmentioning

confidence: 99%

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Skin Necrosis and Purpura Fulminans in Children With and Without Thrombophilia—A Tertiary Center's Experience

Fruchtman

Strauss

Rubinstein

et al. 2015

Pediatric Hematology and Oncology

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“…Platelets (3 μ m) [2] were able to surround the conidia of Aspergillus species but could not ingest them, possibly due to the large size of the fungal elements (2–4 μ m) [1], given that platelets have been shown to fail to ingest large latex spherules (3.13 μ m), although not particles of smaller sizes [12]. Nonetheless, platelets may mount an attack on the fungus by surrounding and covering conidia and hyphae (figure 1).…”

Section: Discussionmentioning

confidence: 99%

“…Host defenses against molds involve innate, humoral, and cell-mediated immunity, and polymorphonuclear leukocytes (PMNs) and macrophages play a dominant role in protecting against asper gillosis, although the contribution of each appears to be site specific [1]. Platelets are the smallest corpuscular components of human blood and, under normal physiological conditions, number from 150 × 10 9 to 400 × 10 9 cells/L [2]. They originate in bone marrow and derive from cytoplasmic fragmentation of a giant precursor cell, the megakaryocyte.…”

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confidence: 99%

Human Platelets AttenuateAspergillusSpecies via Granule‐Dependent Mechanisms

Perkhofer¹,

Kehrel²,

Dierich³

et al. 2008

J INFECT DIS

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Using laser scanning microscopy, we investigated whether platelets are capable of internalizing Aspergillus conidia and examined Aspergillus-platelet adherence. The influence of platelets on fungal growth was evaluated by assessing galactomannan (GM) release, hyphal elongation, and colony size. A secretion assay with [3H]-serotonin (5-hydroxytryptamine [5-HT]) was performed. Exposure to platelets resulted in significantly decreased GM release (P<.05), hyphal elongation (P<.001), colony size, pig-mentation, and 5-HT release (P<.05). A lack of antifungal effects was observed with the microfilament inhibitor cytochalasin D. Platelets attenuate the virulence of Aspergillus species in vitro on the basis of granule-dependent effects.

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