Increased red cell distribution width in Fanconi anemia: a novel marker of stress erythropoiesis

Abstract

BackgroundRed cell distribution width (RDW), a classical parameter used in the differential diagnosis of anemia, has recently been recognized as a marker of chronic inflammation and high levels of oxidative stress (OS). Fanconi anemia (FA) is a genetic disorder associated to redox imbalance and dysfunctional response to OS. Clinically, it is characterized by progressive bone marrow failure, which remains the primary cause of morbidity and mortality. Macrocytosis and increased fetal hemoglobin, two indicators of bone marrow stress erythropoiesis, are generally the first hematological manifestations to appear in FA. However, the significance of RDW and its possible relation to stress erythropoiesis have never been explored in FA. In the present study we analyzed routine complete blood counts from 34 FA patients and evaluated RDW, correlating with the hematological parameters most consistently associated with the FA phenotype.ResultsWe showed, for the first time, that RDW is significantly increased in FA. We also showed that increased RDW is correlated with thrombocytopenia, neutropenia and, most importantly, highly correlated with anemia. Analyzing sequential hemograms from 3 FA patients with different clinical outcomes, during 10 years follow-up, we confirmed a consistent association between increased RDW and decreased hemoglobin, which supports the postulated importance of RDW in the evaluation of hematological disease progression.ConclusionsThis study shows, for the first time, that RDW is significantly increased in FA, and this increment is correlated with neutropenia, thrombocytopenia, and highly correlated with anemia. According to the present results, it is suggested that increased RDW can be a novel marker of stress erythropoiesis in FA.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-016-0485-0) contains supplementary material, which is available to authorized users.

Psoriasis and Psoriatic Arthritis Cardiovascular Disease Endotypes Identified by Red Blood Cell Distribution Width and Mean Platelet Volume

“…This is demonstrated by increased RDW with age as well as increased rates of hematologic malignancies with higher MPV and RDW which are considered negative independent prognostic factors of survival in myelofibrosis and myelodysplasia [45,51]. In fact Sousa et al, advocated RDW as a novel marker of stress erythropoiesis, which may be induced by systemic inflammatory conditions such as psoriasis [52]. Several abnormalities in bone marrow stem cells of psoriatic patients have been previously described, due to a modification of bone marrow microenvironment based on a psoriasis-related pro-inflammatory skin [17].…”

Relationship between red cell distribution width and prognosis in patients with breast cancer after operation: a retrospective cohort study

“…The mechanism that could explain the relation between RDW and survival or disease activity is not clear, but it is considered that high RDW is caused by chronic inflammation, poor nutritional status, oxidative stress, and age-related diseases that lead to changes in erythropoiesis [26,27]. As we all know that inflammation in the rumor microenvironment promotes tumor growth, invasion, angiogenesis, and eventually metastasis.…”

Red blood cell distribution width and ischaemic stroke

“…RDW would increase because of the release of immature red blood cells into the blood stream in certain haematological diseases. 3–5 RDW increases in iron deficiency anaemia, and such increase would appear earlier than the decline of mean corpuscular volume (MCV), which can be an early indication of iron deficiency. When MCV is low, RDW would increase more significantly.…”