QUIZ 3

• Toxic granulation: a condition in which prominent dark granulation either fine or heavy can be observed in band and segmented neutrophils or monocyte. They are azurophilic granules, peroxidase positive.
• Dohle bodies: these inclusion bodies are seen as single or multiple light blue staining inclusions on wright-stained blood smear. They are usually near the periphery of the cytoplasm. These inclusions are prominently seen in neutrophils although they may be seen in monocytes or lymphocytes .
• Pelger-Huet Anomaly: This is genetically acquired autosomal dominant disorder that produces hypo segmentation of many of the mature neutrophils. The nuclear shape may resemble a dumb bell or a pair of eyeglasses.
• May-Hegglin anomaly: This genetic condition is characterized by the presence of Döhle body like inclusions in neutrophils, eosinophils, and monocytes. Abnormally large and poorly granulated platelets and thrombocytopenia frequently coexist in this condition.
• Chediak-Higashi syndrome: this rare disorder is a hereditary disease. It is primarily seen in children and young adults and is characterized by very large granules. This gigantic peroxidase positive deposits represent abnormal lysosomal development in neutrophils and other leukocytes such as monocytes and lymphocytes. Neutrophils display impaired chemotaxis and delay killing of ingested bacteria.
• Alder-Reily inclusion: These purple red particles are precipitated leukopolysaccharides seen primarily in neutrophils eosinophils and basophils. Occasionally they are seen in monocytes and lymphocytes. This inclusions can resemble very course toxic granulation. They are most commonly seen in patients with curler hunter……
• Chronic granulomatous disease: The most serious disorder related to a defect in microbicidal activity. It consists of a group of genetically disorders in which neutrophils and monocytes ingest but cannot kill catalase positive microorganisms such as S. aures, gram positive enteric bacteria, and various fungi especially aspergillus.
• Myeloperoxidase Deficiency (Alius-Grignaschi anomaly): It is a benign inherited disorder that is usually transmitted by autosomal recessive genes. These disorders are manifested by the absence of MP2 enzyme from neutrophils and monocyte not not eosinophils.
• Lactoferrin: It is a rare disorder where specific granules are reduced in quantity and almost devoid of the specific granule protein. This deficiency causes several dysfunctions including unresponsiveness to chemotactic signals and diminish adhesiveness to surfaces of corticos. This deficiency leads to pathogenic infections particularly deep-seated skin abscess.
• Gaucher disease: This inherited disease is caused by the disturbance in the cellular lipid metabolism. The disorder represents a deficiency of beta glucocerebrosidase, the enzyme that normally splits glucose from its parent sphingolipid glucosylceramide. The result of this enzyme deficiency cerebroside accumulates in macrophages or histiocytes.
• Niemann-pick disease: This is similar to Gaucher disease because it is also an inherited abnormality of lipid metabolism. It affects infants and children. The patient average age of death is 5 years. This disorder represents a deficiency of the enzyme that normally cleaves phosphorite coly from its parent sphingolipid. Sphingomyelin accumulates in the tissue macrophages. Its cytoplasm has a foamy appearance.
• Wiskott-Aldrich syndrome: It is classified as a combined immunodeficiency. It is a rare x linked disease caused by 1 of more than 400 mutations in the WAS gene which results in decrease levels of WAS protein.
• Bruton's tyrosine kinase or X-linked agammaglobulinemia (XLA): It is a primary immunodeficiency disease characterized by reductions in all serum immunoglobulin isotypes and profoundly decreased or absent B cells.
• Mucopolysaccharidosis: it is a family of inherited disorder of mucopolysaccharide or glycosaminoglycan degradation.