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Mitochondrial disorders (MIDs) are an increasingly recognised group of multisystem metabolic disorders, which manifest clinically as either syndromic forms or non-syndromic entities.Genetically MIDs are due to mutations in either mtDNA or nDNA located genes encoding for subunits of respiratory chain complexes, ancilliary proteins of respiratory chain complexes, tRNAs, rRNAs, proteins involved in the mtDNA replication machinery, the co-enzyme-Q pathway, the mitochondrial transport machinery, or the mitochondrial biosynthesis.MIDs most frequently manifest in the central nervous system, the peripheral nervous system, the endocrine system, the heart, the liver, or the kidneys.More rarely, they manifest in the gastro-intestinal tract, the cartilage, the integument, or blood cells, Syndromic forms with hematological manifestations include the Pearson syndrome (pancytopenia), Keams-Sayre syndrome (anemia),Barth syndrome (neutropenia), the autosomal recessive mitochondrial myopathy, lactic acidosis and sideroblastic anemia (MLASA) syndrome.In single cases with Leigh syndrome, MERRF-syndrome, and Lebers hereditary optic neuropathy, anemia has been described.Anemia, leukopenia, thrombocytopenia, eosinophilia, or pancytopenia, have been also reported in non-syndromic MIDs with or without affection of other tissues.We aimed to assess how often blood cell abnormalities can be found in our cohort of patients with syndromic and non-syndromic MIDs.We retrospectively reviewed the blood cell counts of patients with syndromic or non-syndromic MIDs which attended the Hospital during the last ten years.Included were only patients in whom abnormal blood cell counts could not be attributed to causes other than a MID.The most frequent hematological abnormality in MIDs was anemia, which was resistant to treatment with iron or vitamins and often required blood transfusions.The second most frequent hematological abnormality was thrombocytopenia, which usually did not impair blood coagulation.Only single patients were found who developed thrombocytosis, leucopenia, or eosinophilia.Except for anemia, the hematological abnormalities found in our MID patients did not require medical treatment.If abnormal blood cell counts cannot be explained by established causes, a MID should be considered, particularly incases with unexplained anemia,thromcyotpenia, thrombocytosis, leucopenia or eosinophilia.Abnormal blood cell counts in MIDs may be the sole manifestation of the MID or, more frequently may be part of a multisystem problem.Hematological abnormalities may be even the initial manifestation of a MID.