Myeloproliferative Disorders (Hematologic Malignancies)

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Although not a malignant neoplasm like other cancers, MPNs are classified within the hematological neoplasms. There are four main myeloproliferative diseases, which can be further categorized by the presence of the Philadelphia chromosome: All MPNs arise from precursors of the myeloid lineages in the bone marrow. The lymphoid lineage may produce similar diseases, the lymphoproliferative disorders acute lymphoblastic leukemia , lymphomas , chronic lymphocytic leukemia and multiple myeloma.

Depending on the nature of the myeloproliferative neoplasm, diagnostic tests may include red cell mass determination for polycythemia , bone marrow aspirate and trephine biopsy, arterial oxygen saturation and carboxyhaemoglobin level, neutrophil alkaline phosphatase level, vitamin B 12 or B 12 binding capacity , serum urate [12] or direct sequencing of the patient's DNA. According to the WHO Classification of Hematopoietic and Lymphoid Neoplasms myeloproliferative neoplasms are divided into categories by diagnostic characteristics as follows: Chronic myeloid leukemia CML with the defining translocation t 9;22 ;Philadelphia chromosome.

What is polycythemia vera?

These disorders are still being revised according to more specific genetic mutations and how often patients end in a fibrotic marrow event. While investigational drug therapies exist, no curative drug treatment exists for any of the MPDs. The goal of treatment for ET and PV is prevention of thrombohemorrhagic complications.

Background

The goal of treatment for MF is amelioration of anemia, splenomegaly, and other symptoms. Low-dose aspirin is effective in PV and ET. Tyrosine kinase inhibitors like imatinib have improved the prognosis of CML patients to near-normal life expectancy. Recently, a JAK2 inhibitor, namely ruxolitinib , has been approved for use in primary myelofibrosis.

From Wikipedia, the free encyclopedia. This article's lead section does not adequately summarize key points of its contents. Please consider expanding the lead to provide an accessible overview of all important aspects of the article. Please discuss this issue on the article's talk page. Rationale and important changes". Somatic mutations of calreticulin in myeloproliferative neoplasms.

N Engl J Med ; N Engl J Med. Bone marrow aspiration and biopsy with cytogenetic studies are required in most, but not all, patients. Cytogenetic studies detect the presence or absence of the Philadelphia chromosome and help to differentiate myeloproliferative disorders from myelodysplastic syndrome. Bone marrow histology shows hypercellularity in most of these disorders.

In the case of myelofibrosis, bone marrow fibrosis is demonstrated on the reticulin stain. Bone marrow fibrosis is also detected in the spent phase of chronic myelogenous leukemia and polycythemia vera. Hematopoietic stem cell transplantation can be considered in young patients with chronic myelogenous leukemia in chronic phase if a human leukocyte antigen HLA -matched donor is available. Approved for use in Philadelphia chromosome—positive chronic myelogenous leukemia patients in chronic phase; also indicated for chronic myelogenous leukemia in blast crisis, accelerated phase, or in chronic phase after interferon-alfa therapy failure; this is the treatment of choice for most patients.

Produces hematologic and molecular remissions in some patients with chronic myelogenous leukemia. When added to interferon alfa, has been reported to increase remission rates. For patients with chronic myelogenous leukemia who are intolerant to interferon-alfa therapy. Indicated for the treatment of adult patients with chronic myeloid leukemia in chronic, accelerated, or myeloid or lymphoid blast phase who are resistant or intolerant to prior therapy including imatinib.

Indicated for the treatment of chronic-phase and accelerated-phase Philadelphia chromosome-positive chronic myelogenous leukemia in adult patients who are resistant or intolerant to prior therapy including imatinib. Treatment for this disease is palliative. Other patients can undergo myelosuppressive therapy with hydroxyurea. Radioactive phosphorous can be used as an alternative therapy in older patients. No curative treatment is available. This usually can be achieved with hydroxyurea or anagrelide. Asymptomatic patients can be monitored clinically until symptomatic.

Hydroxyurea is useful to suppress the number of circulating cells.

Myeloproliferative Neoplasms: Learning Objectives

Patients with painful, massively enlarged spleens refractory to myelosuppressive therapy are occasionally treated with radiation therapy, but they may ultimately require splenectomy. See Treatment and Medication for more detail. Myeloproliferative diseases MPDs are a heterogeneous group of disorders characterized by cellular proliferation of one or more hematologic cell lines in the peripheral blood, distinct from acute leukemia.

According to the French-American-British FAB classification, chronic myeloproliferative diseases consist of the following four diseases:. Open Table in a new window. In some patients, conditions overlap, and clear categorization may be difficult.


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Myeloproliferative disease may evolve into one of the other myeloproliferative conditions, transform to acute leukemia , or both. Some evidence indicates that myeloproliferative diseases arise from malignant transformation of a single stem cell. Involvement of erythropoiesis, neutrophilopoiesis, eosinophilopoiesis, basophilopoiesis, monocytopoiesis, and thrombopoiesis occurs in the chronic phase of CML. Some evidence also indicates that lymphocytes are derived from primordial malignant cells.

Myeloproliferative Disease

This is based on observations that a single isoenzyme for glucosephosphate dehydrogenase G6PD is present in some T and B lymphocytes in women with CML who are heterozygous for isoenzymes A and B. Data from glucosephosphate dehydrogenase G6PD studies, cytogenetic analyses, and molecular methods have established the clonal origin of myeloproliferative diseases; this clonality potentially occurs at different stem cell levels. An attribute common to these disorders appears to be an acquired activating mutation in the gene coding for various tyrosine kinases.

In chronic myelogenous leukemia, the tyrosine kinase activity of the bcr-abl hybrid gene is increased. In polycythemia vera, essential thrombocythemia, and myelofibrosis see the following images , the prevalent genetic lesion appears to be a valine to phenylalanine substitution at amino acid position VF within the Janus kinase 2 JAK2 gene.

American Society of Hematology

At least in myelofibrosis patients the leukemic transformation is probably not related to JAK-2 VF mutation status. A study by Anand et al found that JAK2 mutations generate expansion of later myeloid differentiation compartments, in which homozygous expression of the mutation confers an added proliferative advantage at the single-cell level. The findings suggest that JAK2 inhibitors may control myeloproliferation; however, they may have limited efficacy in eradicating leukemic stem cells. Systemic mastocytosis has been linked with the D mutation of the KIT gene.

On clonal analyses, CALR mutations were found in the earliest phylogenetic node, a finding consistent with its role as a possible initiating mutation.

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The incidence of polycythemia vera in the United States is approximately cases per 1 million population per year. True incidences of essential thrombocythemia and myelofibrosis are not known because epidemiological studies on these disorders are inadequate. The incidence of polycythemia vera is 0. Essential thrombocythemia has an incidence of 0. Myelofibrosis has an international incidence of 0.

CML appears to affect all races with approximately equal frequency. In a study from northern Israel, the incidences of polycythemia vera, essential thrombocythemia, and myelofibrosis in Ashkenazi Jews were fold higher than in Sephardic Jews, and fold higher than in Arabs.


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The female-to-male ratio is 1: Most cases encountered in clinical practice are in patients aged years. Myeloproliferative diseases are uncommon in people younger than 20 years and are rare in childhood. Imatinib compared with interferon and low-dose cytarabine for newly diagnosed chronic-phase chronic myeloid leukemia. N Engl J Med. High incidence of myeloproliferative disorders in Ashkenazi Jews in northern Israel. Acquired mutation of the tyrosine kinase JAK2 in human myeloproliferative disorders. A unique clonal JAK2 mutation leading to constitutive signalling causes polycythaemia vera.

Comprehensive review of JAK inhibitors in myeloproliferative neoplasms. JAK2 V and leukemic transformation in myelofirbrosis with myeloid metaplasia. Effects of the JAK2 mutation on the hematopoietic stem and progenitor compartment in human myeloproliferative neoplasms.

Another Piece of the Myeloproliferative Neoplasms Puzzle. Trends in the incidence of chronic Philadelphia chromosome negative Ph- myeloproliferative disorders in the city of Goteborg, Sweden, during