Primary myelofibrosis

A rare myeloproliferative neoplasm characterized by stem-cell derived clonal over proliferation of mature myeloid lineages, such as erythrocytes, leukocytes, and megakaryocytes, with variable degrees of megakaryocyte atypia, associated with reticulin and/or collagen bone marrow fibrosis, osteosclerosis, ineffective erythropoiesis, angiogenesis, extramedullary hematopoiesis, and abnormal cytokine expression.

Hypertension

The presence of chronic increased pressure in the systemic arterial system.


Total: 8

                       


(per page)
PMID (PMCID)
24843243
(4023057)
 MALE
Extramedullary pulmonary hematopoiesis causing pulmonary hypertension and severe tricuspid regurgitation detected by technetium-99m sulfur colloid bone marrow scan and single-photon emission computed tomography/CT.
Ali SZ, Clarke MJ, Kannivelu A, Chinchure D, Srinivasan S.
Korean J Radiol. 2014;15(3):376-80.
We report the case of a 66-year-old man with known primary myelofibrosis, in whom a Technetium-99m sulfur colloid bone marrow scan with single-photon emission computed tomography (SPECT)/CT revealed a pulmonary hematopoiesis as the cause of pulmonary hypertension and severe tricuspid regurgitation.
22958626
(3436631)
 OTHER
Pulmonary hypertension diagnosed by echocardiography during idiopathic myelofibrosis. A case report and a brief review of the literature.
Perrone C, Cartolari R, Lupi B, Morelli S.
Multidiscip Respir Med. 2010;5(4):267-70.
Pulmonary hypertension (PH) is only occasionally reported, although recent studies have suggested an association between PH and myeloproliferative disorders.We present a case of PH diagnosis by echocardiography in a patient affected by idiopathic myelofibrosis with portal hypertension.
19674818
 FEMALE Middle Aged
[Dyspnea secondary to pulmonary hypertension in a patient with splenic myeloid metaplasia].
Haddad F, Anouti S, Maalouly G, Jammal M, Nemnoum R, Kettaneh A.
Rev Med Interne. 2009;30(9):803-5.
There are several possible pathophysiological links between the development of pulmonary hypertension and myelofibrosis with myeloid metaplasia.
15160962
 MALE
Agnogenic myeloid metaplasia with pleural extramedullary leukemic transformation.
Nadrous HF, Krowka MJ, McClure RF, Tefferi A, Lim KG.
Leuk Lymphoma. 2004;45(4):815-8.
Herein we describe a 71-year-old man with newly diagnosed agnogenic myeloid metaplasia who was evaluated for progressively worsening dyspnea, pulmonary hypertension and bilateral pleural effusions.
15167356
 MALE Infant, Newborn
Pulmonary hypertension complicating bone marrow transplantation for idiopathic myelofibrosis.
Shankar S, Choi JK, Dermody TS, Head DR, Bunin N, Iannone R.
J Pediatr Hematol Oncol. 2004;26(6):393-7.
Idiopathic myelofibrosis is a rare hematologic disorder that is occasionally associated with pulmonary hypertension and has been cured with bone marrow transplantation (BMT).
15167356
 MALE Infant, Newborn
Pulmonary hypertension complicating bone marrow transplantation for idiopathic myelofibrosis.
Shankar S, Choi JK, Dermody TS, Head DR, Bunin N, Iannone R.
J Pediatr Hematol Oncol. 2004;26(6):393-7.
The authors describe a critically ill male infant with idiopathic myelofibrosis and subtle findings suggestive of pulmonary hypertension who was treated with BMT after failing to respond to chemotherapy.
9929105
 MIXED_SAMPLE Adult
Pulmonary hypertension in patients with myelofibrosis secondary to myeloproliferative diseases.
Garcia-Manero G, Schuster SJ, Patrick H, Martinez J.
Am J Hematol. 1999;60(2):130-5.
Six patients (four male, two female), with diagnoses of myeloproliferative disease, myelofibrosis (one with polycythemia vera, three with agnogenic myeloid metaplasia, one with unclassified myeloproliferative syndrome, one with essential thrombocytosis), and pulmonary hypertension are presented.
3493936
 MALE Middle Aged
Agnogenic myeloid metaplasia, portal hypertension, and sinusoidal abnormalities.
Roux D, Merlio JP, Quinton A, Lamouliatte H, Balabaud C, Bioulac-Sage P.
Gastroenterology. 1987;92(4):1067-72.
In the pathogenesis of sinusoidal hypertension as it occurs in agnogenic myeloid metaplasia, all the factors mentioned above should probably be taken into consideration.