Hairy cell leukemia natural history

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2], Vamsikrishna Gunnam M.B.B.S [3], James Nasr[4]; Grammar Reviewer: Natalie Harpenau, B.S.[5]

Overview

Most patients with hairy cell leukemia are asymptomatic at the time of diagnosis. If left untreated, most of the patients with hairy cell leukemia will gradually develop anemia, recurrent infections, and bleeding. The complications of hairy cell leukemia are mainly due to bone marrow failure.

Natural History, Complications, and Prognosis

Natural History

• Most patients with hairy cell leukemia are asymptomatic at the time of diagnosis.
• If left untreated, most of the patients with hairy cell leukemia will gradually develop the following:
  • Anemia
  • Recurrent infections
  • Bleeding

Complications

• Common complications of hairy cell leukemia include:
  • Anemia
  • Recurrent infections
  • Major recurrent bleeding
  • Massive splenomegaly which may rupture

Prognosis

• Prognosis is generally good, and the 5-year survival rate of patients with chronic lymphocytic leukemia is approximately 90%.
• Poor prognostic factors for hairy cell leukemia patients include hairy cell leukemia variant, VH4-34 rearrangement, TP53 mutations, large splenomegaly, leukocytosis, and high circulating hairy cell count.
• The table below lists prognostic factors for hairy cell leukemia patients:

Prognostic Factor	Description
Age	Older age at the time of diagnosis is associated with a worse prognosis.
Gender	Female are associated with a worse prognosis when compared to males.
Race	African American individuals are associated with a worse prognosis.
Infections	A recent history of pneumonia or the presence of major infection at the time of diagnosis are associated with a worse prognosis.
Hepatomegaly	The presence of hepatomegaly is associated with a worse prognosis.
Hemoglobin concentration	A hemoglobin concentration of ≤10 g/dl is associated with a poor prognosis.
Reticulocyte count	A reticulocyte count of <1% is associated with a worse prognosis.
Neutrophil count	A neutrophil count ≤350/μl is associated with a worse prognosis.

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