Limits...
Hairy cell leukemia: A decade long experience of North Indian Hematology Center.

Somasundaram V, Purohit A, Aggarwal M, Manivannan P, Mishra P, Seth T, Tyagi S, Mahapatra M, Pati HP, Saxena R - Indian J Med Paediatr Oncol (2014)

Bottom Line: A total of 35 cases were diagnosed during this period of 12 years of which 27 received cladribine and went in to remission.Median follow-up duration was 26 months. 5 (18%) cases had a relapse and all relapsed cases achieved second remission with cladribine; however, there was no case of second malignancy in our cohort.Upon relapse, these patients could be successfully salvaged with cladribine retreatment.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology, All India Institute of Medical Sciences, New Delhi, India.

ABSTRACT

Introduction: Hairy cell leukemia is a rare chronic B-cell disorder that follows an indolent but progressive course. This disorder is characterized by pancytopenia, splenomegaly, bone marrow fibrosis and the presence of atypical lymphoid cells with hairy projections in peripheral blood, bone marrow and spleen. Treatment is mainly with nucleoside analog cladribine, which induces complete remission in up to 85% cases.

Materials and methods: This is a retrospective analysis of Hairy cell Leukemia cases diagnosed and treated in the Department of Hematology, All India Institute of Medical Sciences, New Delhi between 2002 and 2013. Various parameters such as clinical features, laboratory parameters including complete blood cell count, bone marrow findings, cytochemistry, immunophenotyping by flowcytometry or immunohistochemistry, treatment protocol and complications secondary to treatment and relapse were reviewed.

Results: A total of 35 cases were diagnosed during this period of 12 years of which 27 received cladribine and went in to remission. Median follow-up duration was 26 months. 5 (18%) cases had a relapse and all relapsed cases achieved second remission with cladribine; however, there was no case of second malignancy in our cohort.

Conclusion: Cladribine has emerged as the treatment of choice for hairy cell leukemia given that the overwhelming majority of patients achieve long-lasting complete remissions. Upon relapse, these patients could be successfully salvaged with cladribine retreatment.

No MeSH data available.


Related in: MedlinePlus

(a) Peripheral blood-hairy cell with round nucleus, homogenous spongy chromation with abundant pale blue cytoplasm with circumferential hair like projections (Jenner Giemsastain, ×1000) (b) tartarate resistant acid phosphatase stain showing bright red granular cytoplasmic positivity in the leukemic cells.(tartrate-treated acid phosphatase stain, ×1000)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC4264273&req=5

Figure 1: (a) Peripheral blood-hairy cell with round nucleus, homogenous spongy chromation with abundant pale blue cytoplasm with circumferential hair like projections (Jenner Giemsastain, ×1000) (b) tartarate resistant acid phosphatase stain showing bright red granular cytoplasmic positivity in the leukemic cells.(tartrate-treated acid phosphatase stain, ×1000)

Mentions: At presentation, anemia (hemoglobin [Hb] <11 g/dl) was present in 74% with median Hb of 9.0 g/dl (range, 3-14.9). Leucopenia was noted in 48% of cases with median total leucocyte count of 4.2 × 109 /L (range, 0.2-41.65) and mean platelet count was 82 × 109 /L (range, 10-222) with 60% of patients presenting with thrombocytopenia. 23% of patients had leukocytosis at presentation and pancytopenia was present in 28% cases. Monocytopenia was observed in 91% of cases. About 86% of cases showed classical hairy cells in peripheral blood while all cases showed similar cells in bone marrow. 86% of cases showed TRAP positivity [Figure 1]. In two cases where flow cytometric immunophenotyping could not be done because of repeated "dry tap," immunohistochemistry for annexin A1 helped in reaching final diagnosis. Electron microscopy was contributory in all five cases which showed characteristic elongated slender microvilli [Table 2].


Hairy cell leukemia: A decade long experience of North Indian Hematology Center.

Somasundaram V, Purohit A, Aggarwal M, Manivannan P, Mishra P, Seth T, Tyagi S, Mahapatra M, Pati HP, Saxena R - Indian J Med Paediatr Oncol (2014)

(a) Peripheral blood-hairy cell with round nucleus, homogenous spongy chromation with abundant pale blue cytoplasm with circumferential hair like projections (Jenner Giemsastain, ×1000) (b) tartarate resistant acid phosphatase stain showing bright red granular cytoplasmic positivity in the leukemic cells.(tartrate-treated acid phosphatase stain, ×1000)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4264273&req=5

Figure 1: (a) Peripheral blood-hairy cell with round nucleus, homogenous spongy chromation with abundant pale blue cytoplasm with circumferential hair like projections (Jenner Giemsastain, ×1000) (b) tartarate resistant acid phosphatase stain showing bright red granular cytoplasmic positivity in the leukemic cells.(tartrate-treated acid phosphatase stain, ×1000)
Mentions: At presentation, anemia (hemoglobin [Hb] <11 g/dl) was present in 74% with median Hb of 9.0 g/dl (range, 3-14.9). Leucopenia was noted in 48% of cases with median total leucocyte count of 4.2 × 109 /L (range, 0.2-41.65) and mean platelet count was 82 × 109 /L (range, 10-222) with 60% of patients presenting with thrombocytopenia. 23% of patients had leukocytosis at presentation and pancytopenia was present in 28% cases. Monocytopenia was observed in 91% of cases. About 86% of cases showed classical hairy cells in peripheral blood while all cases showed similar cells in bone marrow. 86% of cases showed TRAP positivity [Figure 1]. In two cases where flow cytometric immunophenotyping could not be done because of repeated "dry tap," immunohistochemistry for annexin A1 helped in reaching final diagnosis. Electron microscopy was contributory in all five cases which showed characteristic elongated slender microvilli [Table 2].

Bottom Line: A total of 35 cases were diagnosed during this period of 12 years of which 27 received cladribine and went in to remission.Median follow-up duration was 26 months. 5 (18%) cases had a relapse and all relapsed cases achieved second remission with cladribine; however, there was no case of second malignancy in our cohort.Upon relapse, these patients could be successfully salvaged with cladribine retreatment.

View Article: PubMed Central - PubMed

Affiliation: Department of Hematology, All India Institute of Medical Sciences, New Delhi, India.

ABSTRACT

Introduction: Hairy cell leukemia is a rare chronic B-cell disorder that follows an indolent but progressive course. This disorder is characterized by pancytopenia, splenomegaly, bone marrow fibrosis and the presence of atypical lymphoid cells with hairy projections in peripheral blood, bone marrow and spleen. Treatment is mainly with nucleoside analog cladribine, which induces complete remission in up to 85% cases.

Materials and methods: This is a retrospective analysis of Hairy cell Leukemia cases diagnosed and treated in the Department of Hematology, All India Institute of Medical Sciences, New Delhi between 2002 and 2013. Various parameters such as clinical features, laboratory parameters including complete blood cell count, bone marrow findings, cytochemistry, immunophenotyping by flowcytometry or immunohistochemistry, treatment protocol and complications secondary to treatment and relapse were reviewed.

Results: A total of 35 cases were diagnosed during this period of 12 years of which 27 received cladribine and went in to remission. Median follow-up duration was 26 months. 5 (18%) cases had a relapse and all relapsed cases achieved second remission with cladribine; however, there was no case of second malignancy in our cohort.

Conclusion: Cladribine has emerged as the treatment of choice for hairy cell leukemia given that the overwhelming majority of patients achieve long-lasting complete remissions. Upon relapse, these patients could be successfully salvaged with cladribine retreatment.

No MeSH data available.


Related in: MedlinePlus