Chapter 096. Paraneoplastic Syndromes: Endocrinologic/Hematologic (Part 7)

Hematologic Syndromes: Introduction The elevation of granulocyte, platelet, and eosinophil counts in most patients with myeloproliferative disorders is caused by the proliferation of the myeloid elements due to the underlying disease rather than a paraneoplastic syndrome. The paraneoplastic hematologic syndromes in patients with solid tumors are less well characterized than the endocrine syndromes because the ectopic hormone(s) or cytokines responsible have not been identified in most of these tumors (Table 96-2). The extent of the paraneoplastic syndromes parallels the course of the cancer.Table 96-2 Paraneoplastic Hematologic SyndromesSyndromeProteinsCancersTypicallyAssociated with SyndromeErythrocytosisErythropoietinRenal cancersHepatocarcinomaCerebellar hemangioblastomasGranulocytosisG-CSFLung cancerGM-CSFGastrointestinal cancer ...
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Chapter 096. Paraneoplastic Syndromes: Endocrinologic/Hematologic (Part 7) Chapter 096. Paraneoplastic Syndromes: Endocrinologic/Hematologic (Part 7) Hematologic Syndromes: Introduction The elevation of granulocyte, platelet, and eosinophil counts in mostpatients with myeloproliferative disorders is caused by the proliferation of themyeloid elements due to the underlying disease rather than a paraneoplasticsyndrome. The paraneoplastic hematologic syndromes in patients with solidtumors are less well characterized than the endocrine syndromes because theectopic hormone(s) or cytokines responsible have not been identified in most ofthese tumors (Table 96-2). The extent of the paraneoplastic syndromes parallelsthe course of the cancer. Table 96-2 Paraneoplastic Hematologic SyndromesSyndrome Proteins Cancers Typically Associated with SyndromeErythrocytosis Erythropoietin Renal cancers Hepatocarcinoma Cerebellar hemangioblastomasGranulocytosis G-CSF Lung cancer GM-CSF Gastrointestinal cancer IL-6 Ovarian cancer Genitourinary cancer Hodgkins diseaseThrombocytosis IL-6 Lung cancer Gastrointestinal cancer Breast cancer Ovarian cancer LymphomaEosinophilia IL-5 Lymphoma Leukemia Lung cancerThrombophlebitis Unknown Lung cancer Pancreatic cancer Gastrointestinal cancer Breast cancer Genitourinary cancer Ovarian cancer Prostate cancer Lymphoma Note: G-CSF, granulocyte colony-stimulating factor; GM-CSF,granulocyte-macrophage CSF; IL, interleukin. Erythrocytosis Ectopic production of erythropoietin by cancer cells causes mostparaneoplastic erythrocytosis. The ectopically produced erythropoietin stimulatesthe production of red blood cells (RBC) in the bone marrow and raises thehematocrit. Other lymphokines and hormones produced by cancer cells maystimulate erythropoietin release but have not been proven to cause erythrocytosis. Most patients with erythrocytosis have an elevated hematocrit (>52% inmen; >48% in women) that is detected on a routine blood count. Approximately3% of patients with renal cell cancer, 10% of patients with hepatoma, and 15% ofpatients with cerebellar hemangioblastomas have erythrocytosis. In most cases theerythrocytosis is asymptomatic. Patients with erythrocytosis due to a renal cell cancer, hepatoma, or CNScancer should have measurement of red cell mass. If the red cell mass is elevated,the serum erythropoietin level should then be measured. Patients with anappropriate cancer, elevated erythropoietin levels, and no other explanation forerythrocytosis (e.g., hemoglobinopathy that causes increased O 2 affinity; Chap.58) have the paraneoplastic syndrome. Erythrocytosis: Treatment Successful resection of the cancer usually resolves the erythrocytosis. If thetumor cannot be resected or treated effectively with radiation therapy orchemotherapy, phlebotomy may control any symptoms related to erythrocytosis.