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Benign Disorders of Leukocytes
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Gene L. Gulati, Zoran Gatalica, Bong H. Hyun
May-Hegglin anomaly is a rare autosomal dominant disorder characterized by (a) Döhle bodies-like inclusions in granulocytes and monocytes, (b) poorly granulated giant platelets, and in some cases (c) mild to moderate thrombocytopenia, sometimes with hemorrhagic manifestations. The leukocyte inclusions are larger and more prominent than the Dohle bodies associated with infections, toxic states, pregnancy, and myeloproliferative disorders. Electron microscopic studies reveal that these inclusions consist of spherical particles and randomly distributed rods surrounded by cisternae of rough endoplasmic reticulum. They stain blue with Wright-Giemsa and pink with methyl-green-pyronin, both denoting RNA. Giant platelets are believed to be the result of some defect in megakaryocyte maturation. Thrombocytopenia is often found incidently, as many patients are asymptomatic. However, detailed history does reveal excessive bleeding after tooth extraction, easy bruising, menorrhagia, and unexpected bleeding following surgical procedures in about half of the patients. May-Hegglin anomaly has not been associated with increased susceptibility to infection.
Hematopoietic System
Published in Pritam S. Sahota, James A. Popp, Jerry F. Hardisty, Chirukandath Gopinath, Page R. Bouchard, Toxicologic Pathology, 2018
Kristin Henson, Tanasa Osborne, Gregory S. Travlos
Histopathologic examination of bone marrow is used to assess marrow cellularity, megakaryocyte numbers and morphology, presence of focal lesions such as inflammation or necrosis, estimation of myeloid and erythroid proportions, and assessment of iron stores, especially in conjunction with iron staining. Structural changes to the hematopoietic compartment, including the endosteum, bone, interstitium, adipose tissue, and vasculature, are also assessed by histopathology (Reagan et al. 2011). Cytologic examination of bone marrow smears may be performed to evaluate effects on early progenitor cells, changes in hematopoietic cell maturation or morphology, unexplained increases or decreases in peripheral erythrocyte, leukocyte, or platelet counts, differentiation of effects on erythroid or lymphoid cell lineages, evaluation of changes in peripheral red blood cell indices or morphology, or to further characterize changes in peripheral leukocyte morphology, that is, presence of Döhle bodies (retained aggregates of rough endoplasmic reticulum appearing as blue cytoplasmic inclusions), cytoplasmic basophilia or vacuolation in leukocytes, and reactive lymphocytes (Harvey 2012; Reagan et al. 2011). Bone marrow cytologic examination is not needed for evaluation of appropriate changes in erythroid or myeloid cells and/or megakaryocytes in response to increased erythrocyte turnover, inflammation, or platelet destruction/consumption, or if increases in peripheral neutrophils and/or decreases in eosinophils are appropriately attributable to stress (Reagan et al. 2011). As peripheral blood lymphocyte counts are not reflective of altered lymphopoiesis, alterations in lymphocyte counts do not necessitate marrow smear evaluation (Reagan et al. 2011). Additionally, as decreases in bone marrow cellularity have been shown to be proportional to decreased food consumption or food restriction, changes in bone marrow cellularity concurrent with decreased food consumption or decreased body weight or body weight gain may not require cytologic evaluation (Reagan et al. 2011).
Pseudo Pelger-Huët anomalies as potential biomarkers for acute exposure radiation dose in rhesus macaques (Macaca mulatta)
Published in International Journal of Radiation Biology, 2022
Joshua M. Hayes, John D. Olson, Yuiko Chino, J. Daniel Bourland, J. Mark Cline, Thomas E. Johnson
Some confusion can be experienced when differentiating PPHAs and immature neutrophils. Immature neutrophils remain in the bone marrow through interactions with the chemokine CXCL12 and its receptor CSCR4 (Vietinghoff and Ley 2008). Immature neutrophils are rarely found in the peripheral blood but can escape the bone marrow during an inflammation response. Immature neutrophils in peripheral blood have a band-like morphology as opposed to a multilobed nucleus. In addition, if immature neutrophils enter the peripheral blood in a hypo-segmented form they can be differentiated from PPHAs by the presence of Dohle bodies, fragments of the cells endoplasmic reticulum lost in development. It is likely that progenitor cells giving rise to granulocytes are impacted by radiation dose to produce the PPHA morphology. Progenitor cells are not terminally differentiated and therefore are more radiosensitive than the post-mitotic neutrophils. In addition, other granulocytes (basophils and eosinophils) often present with a bilobed morphology indicating that a common cellular ancestor is being impacted by radiation exposure. As previously stated, basophils and eosinophils are far less common in the peripheral blood under normal physiological conditions, so basophils and eosinophils are not informative biomarkers of radiation dose.
Screening and diagnosis of inherited platelet disorders
Published in Critical Reviews in Clinical Laboratory Sciences, 2022
Alex Bourguignon, Subia Tasneem, Catherine P. Hayward
A bleeding history evaluation for a PFD should also be mindful of syndromic features that suggest a specific disorder (see reviews [1,99–102]). For example, HPS is associated with oculocutaneous albinism, lung fibrosis, and colitis; Jacobsen/Paris-Trousseau syndrome (PTS) is associated with facial dysmorphism, cardiac defects, and mental retardation; MYH9-related disorders are associated with inclusion bodies in neutrophils (Döhle bodies) with or without sensorineural hearing defects, elevation of liver enzymes, nephritis, and/or cataracts; thrombocytopenia with absent radius (TAR) syndrome is associated with absent radii; and WAS is associated with microplatelets, eczema, and susceptibility to infections.
The utility of flow cytometric platelet forward scatter as an alternative to mean platelet volume
Published in Platelets, 2022
David Connor, David Rabbolini, Marie-Christine Morel-Kopp, Kate Fixter, Dea Donikian, Mayuku Kondo, Onki Chan, Susan Jarvis, Walter Chen, Timothy Brighton, Vivien Chen, Christopher Ward, Joanne Joseph
There are currently over 50 disease associated “TIER1” genes associated with platelet disorders [20]. The utility of reliable platelet size determination is not wholly to accurately predict the precise underlying molecular variant, but rather to enable investigators to group disorders descriptive categories, which have diagnostic relevance. Currently, these categories are disorders with giant, large, slightly increased, normal size or slightly smaller platelets [3,4]. Our group has found this to be an invaluable tool for rationalizing phenotypic investigations. For example, in the setting of thrombocytopenia with giant platelets, immunfluorescence staining for neutrophil inclusions (Dohle bodies) is performed to detect/exclude a MYH9-RD. Likewise in this setting, platelet responses to ristocetin by light transmission aggregometry and GPIb/V/IX expression by flow cytometry is pivotal for the evaluation of possible BSS. However, in the setting of thrombocytopenia with autosomal dominant pattern of inheritance and normal-sized platelets (or slightly enlarged or slightly reduced), MYH10 analysis by western blotting is considered for evaluation of a possible RUNX1 variant [21]. Similarly, platelet size classification by MPD has been used by other groups6 to determine the choice of antibodies employed for immunofluorescence labeling of peripheral blood films for the diagnosis of platelet disorders. Considering the turn-around time for NGS results that may vary between centers and range between weeks to months before results can be obtained, it is important for the hemostasis field to identify and develop phenotypic methods in parallel to genetic advancements to assist the treating physician with the patient’s differential diagnosis and subsequent management.