Mast-Cell Leukemia (MCL)

Mast-cell leukemia (MCL), also called tissue-basophil leukemia, is a clonal malignant mast-cell proliferative disease. May arise from systemic mastocytosis (type II/III) or de novo. As advanced mastocytosis, two feature groups: (1) Mast-cell infiltration: hepatosplenomegaly/nodes, bone lesions/bone tenderness/osteolysis; skin infiltration → pruritus, flushing, urticaria pigmentosa (brown macules/papules/nodules), positive dermatographism. (2) Metachromatic cytoplasmic granules contain heparin/hyaluronic acid, release histamine/glycosaminoglycans/kinins/prostaglandins → intractable gastroduodenal ulcers (abdominal pain/hematemesis/melena), headache, facial/limb edema.

Common Symptoms

Macule; bleeding tendency; lymph-node enlargement; dyspnea; melena; abdominal pain; fatigue

Causes and Pathogenesis

Unknown; possibly c-KIT mutation. AML-like pathology/clinical features; mast-cell infiltration of nodes/liver/spleen ± osteolysis; skin infiltration characteristic (urticaria pigmentosa, dermatographism). Cytoplasmic granules release histamine → hyperhistaminemia: flushing/pruritus/bronchospasm/hypotension/syncope/shock; refractory gastroduodenal ulcers with bleeding/perforation; heparin release → bleeding tendency.

Treatment

Drug, supportive therapy

Preventive Measures

No effective prevention; early diagnosis/treatment.

Department to Visit

It is recommended to visit the Hematology department; an appointment can be booked on this platform before the visit.

Warm reminder: The above information is for health science reference only and cannot replace professional medical diagnosis. If you have related symptoms, please seek medical attention at a regular hospital promptly.