17.4 Pathology



Myelopathies


Congenital myelopathies are very rare bone marrow diseases. They manifest initially in small or school age children.



Panmyelopathies


There are a number of hereditary panmyelopathies, i.e., functional disorders of the bone marrow, that affect all 3 cell types (red and white cells as well as the thrombocytes). The most frequent are summarized as follows.

The most frequent diseases of all three cell lines:

Meer info

Overview of all congenital disorders of the bone marrow.



Blood clotting disorders


Congenital blood clotting disorders are caused by a genetic defect of the megakaryocytes (precursor of thrombocytes) or the plasmatic coagulation factors.



Thrombocytopenias


In the thrombocytopenias one distinguishes between two autosomal recessive hereditary forms:

  • Amegakaryocytic thrombopenia
  • Thrombocytopenia with radius aplasia


Hereditary coagulopathies


Today one knows many differing coagulopathies. Most of them are inherited autosomal recessively. The only exceptions are hemophilias A and B, which are X chromosomal recessively inherited.

Meer info

Overview of heredity, frequency, symptoms and therapy (German).



Disorders of the lymph vessels


Primary lymphatic edema is subdivided into 3 groups:

  • Congenital lymphatic edema is present already at birth: Milroy disease
  • Somewhat later (mostly in adolescence but before age 35) lymphatic edema praecox commences: Meige disease
  • Lymphatic edema tarda begins only after age 35.

Meer info

Overview of the 3 forms.



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