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Primary Myelofibrosis

 Syn. PMF , Chronic idiopathic myelofibrosis , CIMF

Signs & Symptoms

 Enlarged spleen
Note: due to extramedullary hematopoiesis, due to PMF
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis] Harrison [ 735] Cecil [ 492]
Dyspnea
Note: due to Anemia
Ref: Cecil [ 492]
Progressive fatigue
Ref: Cecil [ 492]
Severe fatigue
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis] Harrison [ 736] Cecil [ 492]

Physical Examination

 Enlarged spleen
Note: due to extramedullary hematopoiesis, due to PMF
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis] Harrison [ 735] Cecil [ 492]
Hepatomegaly
Note: due to extramedullary hematopoiesis, due to PMF
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis] Harrison [ 736]
Palpable hepatomegaly
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis]

Lab / Serology

 Poikilocytosis
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis]
Anemia
Note: due to bone marrow failure
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis] Harrison [ 736] Cecil [ 492]
Anisocytosis
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis]

Imagin Study

 Bone marrow fibrosis
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis] Harrison [ 736]
Hepatomegaly
Note: due to extramedullary hematopoiesis, due to PMF
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis] Harrison [ 736]
Mottled appearance of the bone
Note: due to Osteosclerosis
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis]

Complication

 Pulmonary hypertension
Note: in patients with AMM
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis] Harrison [ 736]
Spleen lower border extending below the pelvic brim
Note: Massive splenomegaly
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis]
Spleen right border extending across the midline
Note: Massive splenomegaly
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis]

Risk Factors

 Elder
Ref: Cecil [ 492]

Cause

 JAK2 mutation
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis] Harrison [ 736]
CALR mutation
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis] Harrison [ 735]
MPL mutation
Ref: Uptodate [ Clinical manifestations and diagnosis of primary myelofibrosis] Harrison [ 736]

Other