alder-Reilley bodies are seen in -
Ans. is 'a' i.e., Mucopolysacharidosis Hypersegmentationo The presence of abnormally increased nuclear lobulation, is one of the first hematologic abnormalities seen in megaloblastic anemia.o Normal mature circulating neutrophils have an average of 3 lobes and always fewer than 5 lobes. Eosinophils have fewer than 4 lobes and basophils have fewer than 3 lobes.o More than 3 cells having 5 lobes or a single cell with 6 lobes found in the course of a 100 cell differential is evidence of hypersegementation.o Hypersegmentation is sometimes referred to as a myeloid "right shift". Hypersegmentation may accompany other disorders in which there is a disturbance of maturation, such as iron defiency.Pelger-Huet anomalyo A congenital autosomal dominant disorder in which granulocyte nuclei fail to segment normally, o In the homozygote state the nucleus is round. In heterozygotes most granulocytes have bilobed nuclei ("pince- nez" cells) resembling bands.o The trait is benign and occurs in 1 in 6,000 people. Cell function is normal.o An acquired or pseudo-Pelger-Huet anomaly is seen in myelodysplastic disorders and following drug therapy and may accompany leukemia and certain infections.Toxic granulationo Toxic granulation is found severe inflammatory states.o The toxic granules are azurophilic, usually found in the promyelocyte, metamyelocyte, band and segmented stages.o The toxic granulation is thought to be due to impaired cytoplasmic maturation, in the effort to rapidly generate large numbers of granulocytes.L Dohie bodieso L Dohie bodies are single or multiple blue cytoplasmic inclusions.o They represent remnants of rough endoplasmic reticulum from earlier maturational stages.o They are associated with myeloid "left shifts" and are seen in conjuction with toxic granulation.May-Hegglin anomalyo May-Hegglin anomaly is a rare autosomal dominant abnormality characterized by large pale basophilic inclusions resembling Dohie bodies and appear to be altered RNA.o Giant platelets and sometimes thrombocytopenia are associated with this. The anomaly is usually but may be associated with bleeding.The chedlak-Hagashi syndromeo The chedlak-Hagashi syndrome is a rare autosomal recessive condition associated with abnormally large leukocyte granules resulting from fusion of lysozymes.o This disorder may affect granulocytes, leukocytes and monocytes. Chemotaxis and phagocytosis is defective.o Platelets lack dense granules and platelet function is abnormal.o Giant melanosomes in occular and skin tissues result in hypopigmentation.The alder-reillv anomalyo The alder-reilly anomaly is associated with the genetic mucopoly-saccharidoses.o Patients with mucopolysaccharidoses lack the lysozymal enzymes necessary to break down mucopolysaccharides.o Dense azurophilic granules, resembling toxic granulation in neutrophils, are seen in all leukocytes.o Most characteristic of these disorders are the metachromatic granules surrounded by a clear zone seen in lymphocytes.Alder Reiilv granuleso Alder Reilly granules are larger than normal azurophilic and basophilic granules (Alder-Reilley bodies) in the cytoplasm of granulocytes, monocytes and lymphocytes seen in patients with mucopolysaccharodosis, mucopolysulfatidosis and lipofuscinosis.o Alder reilley granules stain iliac with wright giemsa and are metachromatic with toluidene blue. They are evenly distributed throughout the cytoplasm and at times may be so dense as to partially obscure the nuclear.
Mucopolysacharidosis
Langerhans Histiocytosis
Alport's syndrome
Chediak-Higashi syndrome
Disorder of Metabolism & Connective Tissue