Kosin Med J.  2018 Dec;33(3):438-445. 10.7180/kmj.2018.33.3.438.

Fludarabine and Rituximab in Relapsed or Refractory Hairy Cell Leukmia Variant: A Case Report and Review of Literature

• Lee JW
• Choi YM
• Yang JY
• Han SM
• Kim BS
• Nam SH

• KMID: 2442557
• DOI: http://doi.org/10.7180/kmj.2018.33.3.438

Abstract

Hairy cell leukemia (HCL) is a rare chronic B cell leukemia morphologically characterized by cells with an abundant cytoplasm and hair-like projections that can be found in the peripheral blood and bone marrow. The treatment for HCL is splenectomy or chemotherapy with the purine analogs pentostatin and cladribine. However, patients continue to relapse. Retreatment with the same or alternate purine analogs produces lower response rates and a shorter duration of response. Fludarabine is another purine analog widely used in treating indolent lymphoid cancers, often in combination with rituximab. Here, we report a case of HCL variant in a 60-year-old man who experienced multiple relapses after splenectomy and retreatment with cladribine. The patient was then treated with fludarabine and rituximab combination chemotherapy. After the treatment, he achieved complete remission that continued for 35 months.

Keyword

Cladribine; Fludarabine; Hairy cell leukemia; Rituximab

MeSH Terms

Bone Marrow
Cladribine
Cytoplasm
Drug Therapy
Drug Therapy, Combination
Humans
Leukemia, B-Cell
Leukemia, Hairy Cell
Middle Aged
Pentostatin
Recurrence
Retreatment
Rituximab*
Splenectomy
Cladribine
Pentostatin
Rituximab

Figure

• 

  Fig. 1 Bone marrow aspiration and biopsy. This figure depicts the characteristic malignant cell in the peripheral blood and bone marrow from a patient with hairy cell leukemia. Hairy cells with radial projections from their surface are noted in peripheral blood smear (A,B) and bone marrow aspirate (C,D) with Wright's stain. (×1000) The bone marrow biopsy section shows a subtle infiltration of hairy cell leukemia (E,F). Small and reniform and cleaved nuclei are present, surrounded by ample amounts of clear cytoplasm. And focal fibrosis are noted (H&E, ×100, ×400)

• 

  Fig. 2 The blood counts according to treatments. After splenectomy, platelet recovered within normal range. After first relapse, first course of cladribine chemotherapy showed complete remission(CR) criteria. After second relapse, second course of cladribine chemotherapy also showed complete remission(CR). But the patient experienced myelosuppression due cladribine chemotherapy. After disease free period of 24 months, relapse is revealed by bone marrow biopsy. Fludarabine and rituximab chemotherapy revealed complete remission(CR) from treatment initiation.

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