Info

tive Diseases

Chronic myelogenous leukemia [Ph chromosome, t(9;22) (q34;q11). BCR-ABLpositive] Chronic neutrophilic leukemia

Chronic eosinophilic leukemia (hypereosinophilic syndrome) Polycythemia vera Chronic idiopathic myelofibrosis Essential thrombocythemia Chronic myeloproliferative disease unclassifiable

Chronic myelogenous leukemia [Ph chromosome, t(9;22) (q34;q11). BCR-ABLpositive] Chronic neutrophilic leukemia

Chronic eosinophilic leukemia (hypereosinophilic syndrome) Polycythemia vera Chronic idiopathic myelofibrosis Essential thrombocythemia Chronic myeloproliferative disease unclassifiable

Adapted from: Jaffee ES, Harris NL, Stein H, Vardiman JW, eds. World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Haematopoietic Lymphoid Tissues. Lyon: IARC Press; 2001.

Other common features shared by these disorders are splenomegaly, hepatomegaly, increased leukocytosis, thrombocytosis, and erythrocytosis. There may be various degrees of bone marrow fibrosis. All the CMPDs have interrelationships between the disorders. Transitions are common between disorders and many finally terminate into an acute myelogenous leukemia (AML).1 Review Figure 12.1 for these interrelationships. A very small percentage of CMPDs can terminate into an acute lymphoblastic leukemia (ALL). An increase in the percentage of blasts in the peripheral blood and bone marrow indicates the onset of an accelerated stage or transformation to an acute process.

The CMPDs are primarily diseases of adults. The peak onset is in the fifth to the seventh decades of life.1 The major clinical and pathological findings are the unregulated proliferation of cells in the bone marrow. This results in the increased numbers of mature cells in peripheral blood. The increase is found in the granu-locytes, usually the neutrophils, platelets, or RBCs. These disorders typically manifest a normocytic, normochromic anemia with all three cell lines involved. The dysfunction in the CMPDs is a loss of regulatory signals that control the production of the mature cells. One of the important bone marrow findings that overlap the various CMPDs is marrow fibrosis. Fibrosis is defined as the replacement of normal bone marrow elements with connective tissue. Classification of these diseases is based on the lineage of the predominant cell present, marrow fibrosis, and clinical and pathological findings.

Table 12.2 O Characteristics of Chronic Myeloproliferative Disorders

Bone Philadelphia

Marrow Chromosome Organ CMPD Cell Line WBC Fibrosis (Ph1) Involvement

Chronic myelogenous leukemia (CML)

Polycythemia vera (PV)

Myelofibrosis with myeloid metaplasia (MMM)

Myeloid

Erythroid, myeloid megakaryocyte Teardrop, erythrocytes Fibroblasts

Essential thrombocythemia (ET) Megakaryocyte

Increased Variable Present

Increased None Absent

Variable Increased Absent

Normal None Absent

Splenomegaly Hepatomegaly

Splenomegaly Hepatomegaly Splenomegaly Hepatomegaly

Splenomegaly

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