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A myelodysplastic syndrome (MDS) is one of a group of cancers in which immature blood cells in the bone marrow do not mature, and as a result, do not develop into healthy blood cells. Early on, no symptoms typically are seen. Later, symptoms may include fatigue, shortness of breath, bleeding disorders, anemia, or frequent infections. Some types may develop into acute myeloid leukemia.
In oncology, polycythemia vera (PV) is an uncommon myeloproliferative neoplasm in which the bone marrow makes too many red blood cells. The majority of cases are caused by mutations in the JAK2 gene, most commonly resulting in a single amino acid change in its protein product from valine to phenylalanine at position 617.
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired, life-threatening disease of the blood characterized by destruction of red blood cells by the complement system, a part of the body's innate immune system. This destructive process occurs due to deficiency of the red blood cell surface protein DAF, which normally inhibits such immune reactions. Since the complement cascade attacks the red blood cells within the blood vessels of the circulatory system, the red blood cell destruction (hemolysis) is considered an intravascular hemolytic anemia. There is ongoing research into other key features of the disease, such as the high incidence of venous blood clot formation. Research suggests that PNH thrombosis is caused by both the absence of GPI-anchored complement regulatory proteins on PNH platelets and the excessive consumption of nitric oxide (NO).
Polycythemia is a laboratory finding in which the hematocrit and/or hemoglobin concentration are increased in the blood. Polycythemia is sometimes called erythrocytosis, and there is significant overlap in the two findings, but the terms are not the same: polycythemia describes any increase in hematocrit and/or hemoglobin, while erythrocytosis describes an increase specifically in the number of red blood cells in the blood.
A megakaryocyte is a large bone marrow cell with a lobated nucleus that produces blood platelets (thrombocytes), which are necessary for normal clotting. In humans, megakaryocytes usually account for 1 out of 10,000 bone marrow cells, but can increase in number nearly 10-fold during the course of certain diseases. Owing to variations in combining forms and spelling, synonyms include megalokaryocyte and megacaryocyte.
Chronic myelogenous leukemia (CML), also known as chronic myeloid leukemia, is a cancer of the white blood cells. It is a form of leukemia characterized by the increased and unregulated growth of myeloid cells in the bone marrow and the accumulation of these cells in the blood. CML is a clonal bone marrow stem cell disorder in which a proliferation of mature granulocytes and their precursors is found; characteristic increase in basophils is clinically relevant. It is a type of myeloproliferative neoplasm associated with a characteristic chromosomal translocation called the Philadelphia chromosome.
Tumors of the hematopoietic and lymphoid tissues or tumours of the haematopoietic and lymphoid tissues are tumors that affect the blood, bone marrow, lymph, and lymphatic system. Because these tissues are all intimately connected through both the circulatory system and the immune system, a disease affecting one will often affect the others as well, making aplasia, myeloproliferation and lymphoproliferation closely related and often overlapping problems. While uncommon in solid tumors, chromosomal translocations are a common cause of these diseases. This commonly leads to a different approach in diagnosis and treatment of hematological malignancies. Hematological malignancies are malignant neoplasms ("cancer"), and they are generally treated by specialists in hematology and/or oncology. In some centers "hematology/oncology" is a single subspecialty of internal medicine while in others they are considered separate divisions. Not all hematological disorders are malignant ("cancerous"); these other blood conditions may also be managed by a hematologist.
In hematology, thrombocythemia is a condition of high platelet (thrombocyte) count in the blood. Normal count is in the range of 150×109 to 450×109 platelets per liter of blood, but investigation is typically only considered if the upper limit exceeds 750×109/L.
In hematology, essential thrombocythemia (ET) is a rare chronic blood cancer characterised by the overproduction of platelets (thrombocytes) by megakaryocytes in the bone marrow. It may, albeit rarely, develop into acute myeloid leukemia or myelofibrosis. It is one of the blood cancers wherein the bone marrow produces too many white or red blood cells, or platelets.
Primary myelofibrosis (PMF) is a rare bone marrow blood cancer. It is classified by the World Health Organization (WHO) as a type of myeloproliferative neoplasm, a group of cancers in which there is activation and growth of mutated cells in the bone marrow. This is most often associated with a somatic mutation in the JAK2, CALR, or MPL genes. In PMF, the bony aspects of bone marrow are remodeled in a process called osteosclerosis; in addition, fibroblast secrete collagen and reticulin proteins that are collectively referred to as (fibrosis). These two pathological processes compromise the normal function of bone marrow resulting in decreased production of blood cells such as erythrocytes, granulocytes and megakaryocytes, the latter cells responsible for the production of platelets.
Myeloproliferative neoplasms (MPNs) are a group of rare blood cancers in which excess red blood cells, white blood cells or platelets are produced in the bone marrow. Myelo refers to the bone marrow, proliferative describes the rapid growth of blood cells and neoplasm describes that growth as abnormal and uncontrolled.
Anagrelide is a drug used for the treatment of essential thrombocytosis, or overproduction of blood platelets. It also has been used in the treatment of chronic myeloid leukemia.
Acute erythrocyte leukemia(AEL) is an extremely rare form of acute myeloid leukemia (less than 1% of AML cases) which is characterized by neoplastic proliferation of erythroid cells with features of maturation arrest (increased erythroblasts) and high prevalence of biallelic TP53 alterations. It is defined as type "M6" under the FAB classification.
GATA-binding factor 1 or GATA-1 is the founding member of the GATA family of transcription factors. This protein is widely expressed throughout vertebrate species. In humans and mice, it is encoded by the GATA1 and Gata1 genes, respectively. These genes are located on the X chromosome in both species.
Chronic neutrophilic leukemia (CNL) is a rare myeloproliferative neoplasm that features a persistent neutrophilia in peripheral blood, myeloid hyperplasia in bone marrow, hepatosplenomegaly, and the absence of the Philadelphia chromosome or a BCR/ABL fusion gene.
The thrombopoietin receptor also known as the myeloproliferative leukemia protein or CD110 is a protein that in humans is encoded by the MPL oncogene.
Acute megakaryoblastic leukemia (AMKL) is life-threatening leukemia in which malignant megakaryoblasts proliferate abnormally and injure various tissues. Megakaryoblasts are the most immature precursor cells in a platelet-forming lineage; they mature to promegakaryocytes and, ultimately, megakaryocytes which cells shed membrane-enclosed particles, i.e. platelets, into the circulation. Platelets are critical for the normal clotting of blood. While malignant megakaryoblasts usually are the predominant proliferating and tissue-damaging cells, their similarly malignant descendants, promegakaryocytes and megakaryocytes, are variable contributors to the malignancy.
Acute panmyelosis with myelofibrosis (APMF) is a poorly defined disorder that arises as either a clonal disorder, or following toxic exposure to the bone marrow.
Ruxolitinib, sold under the brand name Jakafi among others, is a medication used for the treatment of intermediate or high-risk myelofibrosis, a type of myeloproliferative neoplasm that affects the bone marrow; polycythemia vera, when there has been an inadequate response to or intolerance of hydroxyurea; and steroid-refractory acute graft-versus-host disease. Ruxolitinib is a Janus kinase inhibitor. It was developed and marketed by Incyte Corp in the US under the brand name Jakafi, and by Novartis elsewhere in the world, under the brand name Jakavi.
Carcinocythemia, also known as carcinoma cell leukemia, is a condition in which cells from malignant tumours of non-hematopoietic origin are visible on the peripheral blood smear. It is an extremely rare condition, with 33 cases identified in the literature from 1960 to 2018. Carcinocythemia typically occurs secondary to infiltration of the bone marrow by metastatic cancer and carries a very poor prognosis.
References
- 1 2 3 4 5 Finazzi, Guido; Vannucchi, Alessandro M.; Barbui, Tiziano (2018-11-07). "Prefibrotic myelofibrosis: treatment algorithm 2018". Blood Cancer Journal. 8 (11): 104. doi:10.1038/s41408-018-0142-z. ISSN 2044-5385. PMC 6221891 . PMID 30405096.
- ↑ Arber, Daniel A.; Orazi, Attilio; Hasserjian, Robert; Thiele, Jürgen; Borowitz, Michael J.; Le Beau, Michelle M.; Bloomfield, Clara D.; Cazzola, Mario; Vardiman, James W. (2016-05-19). "The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia". Blood. 127 (20): 2391–2405. doi: 10.1182/blood-2016-03-643544 . ISSN 0006-4971. PMID 27069254.
- ↑ Barbui, Tiziano; Thiele, Juergen; Gisslinger, Heinz; Orazi, Attilio; Vannucchi, Alessandro M.; Gianelli, Umberto; Beham-Schmid, Christine; Tefferi, Ayalew (2019). "Comments on pre-fibrotic myelofibrosis and how should it be managed". British Journal of Haematology. 186 (2): 358–360. doi: 10.1111/bjh.15840 . hdl: 2434/677709 . ISSN 1365-2141. PMID 30847907.
- ↑ Barbui, Tiziano; Thiele, Juergen; Gisslinger, Heinz; Orazi, Attilio; Vannucchi, Alessandro M.; Gianelli, Umberto; Beham-Schmid, Christine; Tefferi, Ayalew (2019). "Comments on pre-fibrotic myelofibrosis and how should it be managed". British Journal of Haematology. 186 (2): 358–360. doi: 10.1111/bjh.15840 . hdl: 2434/677709 . ISSN 1365-2141. PMID 30847907.
- ↑ Rumi, Elisa; Sant’Antonio, Emanuela; Boveri, Emanuela; Pietra, Daniela; Cavalloni, Chiara; Roncoroni, Elisa; Astori, Cesare; Arcaini, Luca (2018-07-03). "Diagnosis and management of prefibrotic myelofibrosis". Expert Review of Hematology. 11 (7): 537–545. doi:10.1080/17474086.2018.1484280. ISSN 1747-4086. PMID 29862872. S2CID 44097453.
- ↑ Barosi, Giovanni; Rosti, Vittorio; Bonetti, Elisa; Campanelli, Rita; Carolei, Adriana; Catarsi, Paolo; Isgrò, Antonina M.; Lupo, Letizia; Massa, Margherita; Poletto, Valentina; Viarengo, Gianluca (2012-04-20). "Evidence that Prefibrotic Myelofibrosis Is Aligned along a Clinical and Biological Continuum Featuring Primary Myelofibrosis". PLOS ONE. 7 (4): e35631. Bibcode:2012PLoSO...735631B. doi: 10.1371/journal.pone.0035631 . ISSN 1932-6203. PMC 3334973 . PMID 22536419.