Histologic criteria for classification and differential diagnosis of chronic myeloproliferative disorders. 1984

B Frisch, and R Bartl, and R Burkhardt, and K Jäger

The chronic myeloproliferative disorders (MPD) are well characterized clinical entities comprising polycythaemia vera (PV), idiopathic thrombocythaemia (IT) (also called megakaryocytic myelosis, mature type; MegM), chronic myeloid leukaemia (CML), and myelofibrosis/osteomyelosclerosis (MF/OMS). Three thousand one hundred and eight bone biopsies of 2629 patients with established MPD were examined to investigate the histologic features of MPD in a large material in order to identify criteria for the histologic classification and differential diagnosis of these disorders. Detailed histologic characteristics were defined for each of the disorders and the results showed that in the majority of cases MPD may be recognized and classified by the initial bone marrow histology. Utilizing the predominant proliferative cell(s) in the bone marrow, PV was categorized into 4 types: 1. the classic, tri-linear type; 2. a bi-linear type with hyperplasia of the erythroid and granulocytic lines; 3. a bi-linear type with hyperplasia of the erythroid and megakaryocytic cell lines and 4. a uni-linear type with isolated increased erythrocytic proliferation. CML showed 2 sub-divisions: 1. the granulocytic, uni-linear type and 2. the bi-linear type with proliferation of myeloid and megakaryocytic lines. The former had a tendency to evolve into blastic crisis, while the latter was prone to develop into MF/OMS. It was primarily uni-linear exhibiting increased megakaryocytes. In most cases, MF/OMS was shown (by means of follow-up biopsies) to arise out of those entities of MPD which had included megakaryocytic hyperplasia and to which the proliferation of fibroblasts was secondary. The conclusion is drawn that an initial bone marrow biopsy provides additional diagnostic and prognostic data in this group of haematologic malignancies.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009196 Myeloproliferative Disorders Conditions which cause proliferation of hemopoietically active tissue or of tissue which has embryonic hemopoietic potential. They all involve dysregulation of multipotent MYELOID PROGENITOR CELLS, most often caused by a mutation in the JAK2 PROTEIN TYROSINE KINASE. Disorder, Myeloproliferative,Disorders, Myeloproliferative,Myeloproliferative Disorder
D001853 Bone Marrow The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells. Marrow,Red Marrow,Yellow Marrow,Marrow, Bone,Marrow, Red,Marrow, Yellow
D003937 Diagnosis, Differential Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures. Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly

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