What causes hematologic paraneoplastic syndromes?

Updated: Jan 12, 2021
  • Author: Luigi Santacroce, MD; Chief Editor: Wafik S El-Deiry, MD, PhD  more...
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Erythrocytosis results from an increase of erythropoietin (EPO), which may occur in response to hypoxia, or may result from ectopic production of EPO or EPO-like substances or from altered catabolism of EPO itself. Erythrocytosis is common in cancers of the liver, kidney, adrenal glands, lung, thymus, and CNS as well as in gynecologic tumors and myosarcomas. It always disappears after removal of the primary tumor.

Anemia is a common presenting symptom of several neoplasms and results from chronic hemorrhages from ulcerated tumors, altered intestinal absorption of vitamins B-6 and B-12, and increased destruction or insufficient production of RBCs. Three types of paraneoplastic anemias may be described, as follows:

  • Chronic anemia resulting from an anti-erythropoietin factor, reduction in mean red blood cell (RBC) life, and poor iron availability

  • Microangiopathic hemolytic anemia resulting from diffuse intravascular coagulation (DIC), with formation of fibrin filaments in capillary vessels and consequent mechanical hemolysis

  • Autoimmune hemolytic anemia resulting from anti-RBC antibodies that can be either produced by lymphomatous clones or directed against novel antigens produced by teratomas and ovarian cystadenocarcinomas

  • DIC is typical of epithelial tumors, leukemias, and lymphomas (in particular, acute promyelocytic leukemia); usually, DIC has a slow and gradual onset, but in some cases, DIC appears in an acute and severe form, characterized by typical thrombotic and/or hemorrhagic manifestations, and sometimes leading to thrombocytopenia

  • Thrombocytopenia may also be caused by autoantibodies; the causes of thrombocytosis are still unknown

  • Marantic endocarditis is typical of mucous adenocarcinomas of lung, stomach, and pancreas

  • Leukemoid reactions are probably caused by mechanical stimulation of bone marrow, resulting from bone metastases, or they may be caused by humoral stimuli resulting from neosynthesized blastic factors or factors released from the foci of tumor necrosis

  • Leukopenia is not common and is essentially related to metastatic compression of bone marrow

  • Gammopathies occurring in cancer patients may be monoclonal (immunoglobulin G [IgG] or the rarer immunoglobulin M [IgM]), monoclonal secondary (IgG + IgM), or polyclonal (IgG); gammopathies probably are related to an antigenic stimulus of the tumor on some immune clones

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