History

Patients with a predisposing condition presents with a history of that disease. There may be a family history or a history of exposure to ionizing radiation.

Clinical symptoms may be mild and can include insidious onset of signs related to marrow dysfunction and resulting cytopenias. Patients may present with pallor and fatigue due to anemia, with bruising or bleeding due to thrombocytopenia. In some patients, fever, weight loss, night sweats, bone pain, and left upper quadrant pain are reported.

Some patients are asymptomatic at the time of presentation, and the condition is identified as an incidental finding during an evaluation of another condition.

A detailed family history may identify an affected relative. Parental consanguinity may be present in autosomal recessive cases.

Physical Examination

Physical findings reflect the cytokine release, marrow malfunction, and extramedullary hematopoiesis. Symptoms may include, but are not limited to, the following:

Pallor (anemia)
Bruising, petechiae, or bleeding (thrombocytopenia)
Splenomegaly (frequent), hepatomegaly, or lymphadenopathy
Stigmata of a predisposing condition

Multiple hemangiomas have been described in 1 affected pair of siblings with idiopathic myelofibrosis (IMF).

Differential Diagnoses

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Media Gallery

Photomicrograph of a peripheral smear of a patient with agnogenic myeloid metaplasia (idiopathic myelofibrosis) shows findings of leukoerythroblastosis, giant platelets, and few teardrop cells.

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Author

Trisha Simone Natanya Tavares, MD Contract Clinical Hematologist and Oncologist, Department of Pediatric Hematology and Oncology, Presbyterian Healthcare; Volunteer Hematology/Oncology Physician, St Vincent de Paul Medical Center

Disclosure: Nothing to disclose.

Coauthor(s)

J Martin Johnston, MD Associate Professor of Pediatrics, Mercer University School of Medicine; Director of Hematology/Oncology, The Children's Hospital at Memorial University Medical Center; Consulting Oncologist/Hematologist, St Damien's Pediatric Hospital

J Martin Johnston, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, International Society of Paediatric Oncology

Disclosure: Nothing to disclose.

Chief Editor

Cameron K Tebbi, MD Director, Children's Cancer Research Group Laboratories

Cameron K Tebbi, MD is a member of the following medical societies: International Society of Pediatric Oncology

Disclosure: Nothing to disclose.

Acknowledgements

James L Harper, MD Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center

James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society

Disclosure: Nothing to disclose.

Sharada A Sarnaik, MBBS Professor of Pediatrics, Wayne State University School of Medicine; Director, Sickle Cell Center, Attending Hematologist/Oncologist, Children's Hospital of Michigan

Sharada A Sarnaik, MBBS is a member of the following medical societies: American Association of Blood Banks, American Association of University Professors, American Society of Hematology, American Society of Pediatric Hematology/Oncology, New York Academy of Sciences, and Society for Pediatric Research

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Pediatric Myelofibrosis Clinical Presentation

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