E Hairy cell Lukemia | Anatomy2Medicine
E Hairy Cell Leukemia Prognosis

E Hairy cell Lukemia

    • Hairy Cell Leukemia
      • B-cell neoplasm (MCQ)
      • median age of 55
      • male-to-female ratio of 5 : 1.
  • Morphology.
  • Hairy cell leukemia derives its picturesque name from the appearance of the leukemic cells, which have fine hairlike projections that are best recognized under the phase-contrast microscope
        • routine peripheral blood smears, hairy cells have
          • round, oblong, or reniform nuclei (MCQ)
  • moderate amounts of pale blue cytoplasm with threadlike or bleblike extensions.
  • “Dry tap” on Bone marrow aspiration (MCQ)
          • Bone marrow is involved by a  diffuse interstitial infiltrate of cells with oblong or reniform nuclei, condensed chromatin, and pale cytoplasm.
          • Because these cells are enmeshed in an extracellular matrix composed of reticulin fibrils, they usually cannot be aspirated (MCQ)
          • They are only seen in marrow biopsies.
        • Spleen
  • The splenic red pulp is usually heavily infiltrated
        • lead to obliteration of white pulp (MCQ)
        • has a beefy red gross appearance(MCQ)
      • Hepatic portal triads are also involved frequently.
    • Immunophenotype
    • Hairy cell leukemias
      • typically express the
  • pan-B-cell markers CD19 and CD20(MCQ)
        • surface Ig (usually IgG) (MCQ)
      • express certain relatively distinctive markers
  • CD11c, CD25, and CD103. (MCQ)
  • Molecular Pathogenesis.
  • Analysis of Ig gene sequences has revealed a high incidence of somatic hypermutation, suggesting a post-germinal center memory B-cell origin. (MCQ)

Clinical Features.

  • result largely from infiltration of the bone marrow, liver, and spleen. (MCQ)
  • Splenomegaly
      • often massive(MCQ)
      • most common and sometimes the only abnormal physical finding.
    • Hepatomegaly
      • less common and not as marked
    • lymphadenopathy is rare
  • Pancytopenia (MCQ)
      • result from marrow involvement and splenic sequestration is seen in more than half the cases.
  • About one third of those affected present with infections
    • There is an increased incidence of atypical mycobacterial infections, possibly related to frequent unexplained monocytopenia.

Clinical course and prognosis

  • follows an indolent course
  • exceptionally sensitive to “gentle” chemotherapeutic regimens, which produce long-lasting remissions. (MCQ)
  • Tumors often relapse after 5 or more years, yet generally respond well when retreated with chemotherapy.
  • The overall prognosis is excellent. (MCQ)