Pelger-Huet anomaly

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Pelger-Huet anomaly Classification and external resources
ICD-10	D72.0
ICD-9	288.2
OMIM	169400
DiseasesDB	29515
eMedicine	ped/1753
MeSH	D010381

Pelger-Huet anomaly is a blood laminopathy associated with the lamin B receptor. It is characterized by a white blood cell type known as a neutrophil whose nucleus is hyposegmented.

It is a genetic disorder with an autosomal dominant inheritance pattern. Heterozygotes are clinical normal although their neutrophils may be mistaken for immature cells which may cause mistreatment in a clinical setting. Homozygotes tend to have neutrophils with rounded nuclei that do have some functional problems.

v • d • e Pathology: hematology, hematologic disease (primarily D50-D77, 280-289) RBCs/anemia/ hemoglobinopathy (Myeloid) nutritional anemia: Iron deficiency anemia, Plummer-Vinson syndrome, Megaloblastic anemia (Pernicious anemia) hereditary hemolytic anemia: enzyme disorders (G6PD Deficiency, Pyruvate kinase deficiency,Triosephosphate isomerase deficiency), Thalassemia, Sickle-cell disease/trait, Hereditary spherocytosis, Hereditary elliptocytosis, Hereditary stomatocytosis acquired hemolytic anemia: Autoimmune (Warm), HUS, MAHA, PNH, PCH aplastic anemia: Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia • Sideroblastic anemia Polycythemia - Methemoglobinemia Coagulation/platelets (Myeloid) coagulopathy: DIC (Congenital afibrinogenemia, Purpura fulminans) • Hemophilia (A/VII, B/IX, C/XI, XIII) • Von Willebrand disease • Hypoprothrombinemia Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP primary hypercoagulable state: Protein C deficiency - Protein S deficiency - Antithrombin III deficiency - Factor V Leiden - Activated protein C resistance - Antiphospholipid syndrome other hemorrhagic conditions: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Grey platelet syndrome - May Hegglin anomaly - Pelger-Huet anomaly Essential thrombocytosis - Thrombocytopenia Monocytes/Macrophages (Myeloid) WHO-I histiocytosis (Langerhans cell histiocytosis) WHO-II/non-Langerhans-cell histiocytosis (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis) WHO-III/malignant histiocytic disorders (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease) -cytosis: Monocytosis -penia: Monocytopenia Granulocytes (Myeloid) -cytosis: granulocytosis (Neutrophilia, Eosinophilia, Basophilia) -penia: Granulocytopenia/agranulocytosis (Neutropenia, Kostmann syndrome) Other/general myeloid Chronic granulomatous disease Lymphoid -cytosis: Lymphocytosis -penia: Lymphopenia Other Splenic disease (Asplenia/hyposplenism, Wandering spleen) - Pancytopenia See also hematological malignancy and immune disorders