The clinico-hematological profile of hairy cell leukaemia: A single centre experience
Department of Hematology, All India Institute of Medical Sciences, New Delhi 110029, India. Hematology (Amsterdam, Netherlands)
(Impact Factor: 1.25).
06/2010; 15(3):162-4. DOI: 10.1179/102453309X12583347113816
The response rates and overall survival of hairy cell leukemia has changed remarkably with cladribine. Twenty patients diagnosed as hairy cell leukemia over a 5 year period were evaluated. Median age of the patients was 52.5 years. Splenomegaly was seen in 85% and hepatomegaly in 50% patients. At presentation, pancytopenia was seen in 45% and bicytopenia in 20% patients. Tartrate resistant acid phosphatase was positive in 73.68%. Cladribine was given in 13 patients. Overall survival was 84.6%. Median duration of follow-up was 17 months (4-108 months). Direct Coombs' test positive autoimmune hemolytic anemia was seen in two patients (15.38%) on cladribine therapy who required treatment with steroids.
Available from: Prabhu Manivannan
- "Absence of splenomegaly in cases of hairy cell leukemia: literature review Incidence of absence of splenomegaly Author reference Number of patients Absence of splenomegaly in percentage (%) Burke JS et al. 1974  2 1 0 Bouroncle et al. 1979  8 2 7 Chatterjee et al. 2008  1 5 0 Bhargava et al. 2010  "
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ABSTRACT: Hairy cell leukemia (HCL) is characterized by
pancytopenia and usually associated with massive splenomegaly,
however the same may not be true in the clinical
settings. Here we report four cases of HCL and all of them
were without the classical clinical feature of splenomegaly.
This is an observational study conducted between January
2013 to March 2014 where we could diagnose ten cases of
HCL in Department of Hematology, All India Institute of
Medical Sciences, New Delhi. Of these, four cases attracted
attention because of absence of classical clinical features
of HCL. Of the four cases, three presented with
weakness/fatigability while fourth patient presented with
recurrent respiratory tract infection. Surprising finding in
these cases was absence of splenomegaly, both clinically
and on imaging which demerit the suspicion of HCL
clinically. All four had bi/pancytopenia and bone marrow
examination coupled with immunophenotypic analysis
confirmed the diagnosis of HCL. Three patients received
chemotherapy with cladribine and achieved complete
hematological remission. One patient did not receive chemotherapy
due to poor general condition and was subsequently
lost to follow up. To conclude, HCL can and do
present without splenomegaly and this should not restrain
one from suspecting HCL based on histomorphology which
needs to be further confirmed by ancillary techniques. This
finding in our series could be because these cases were
picked early in their natural course of the disease. A high
index of suspicion is essential for diagnosing and appropriately
managing such cases
Available from: Nasir Hussain
- "Affected patients may complain of fatigue, easy and prolonged bleeding, easy bruises, frequent infections, and abdominal discomfort. The vast majority of patients with HCL have splenomegaly (prevalence ranges from 66% to 100%)    . Furthermore, splenomegaly tends to be massive in patients with HCL . "
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ABSTRACT: Hairy cell leukemia is a rare lymphoid neoplasm arising from mature B-lymphocytes. Clinically, the disease presents with splenomegaly and abdominal discomfort, frequent infections, fatigue and bleeding because of related cytopenias. Bone marrow biopsy is essential for diagnosis. Below we describe a case of a 70-year-old African-American male who presented to our hematology clinic complaining of fatigue. Clinical exam and computed tomography imaging did not reveal splenic enlargement. Blood work-up revealed pancytopenia and bone marrow was diagnostic for hairy cell leukemia.The patient was started on cladribine, with gradual improvement of his symptoms and blood count abnormalities. Therefore, it is essential to keep hairy cell leukemia in the differential of pancytopenia even in the absence of a splenomegaly.
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ABSTRACT: Hairy cell leukemia (HCL) is a rare, low grade, B-cell neoplasm with a characteristic morphologic and immunophenotypic profile. It has to be distinguished from chronic lymphoproliferative disorders because of different treatment protocol and clinical course.
To evaluate clinicopathological features including immunophenotypic analysis of cases diagnosed as HCL.
The present study included 28 cases diagnosed over a period of nine years (2002-2010). Clinical presentation, complete blood count, bone marrow aspirate, and flow cytometric analysis of cases were reviewed. Treatment and follow-up details (ranging from 3-90 months) were noted.
This study revealed 28 cases (referrals-7, indoor-21), aged 26-69 years with a median age of 47 years, with a male predominance (M:F=6:1). The presenting complaints were weakness (80%) followed by fever (56%) and abdominal pain. Physical examination revealed splenomegaly in most patients (92%) and hepatomegaly in a minority (28%). The common laboratory features were anemia in 23 cases, pancytopenia in 14 cases, while two patients had leukocytosis and three patients had normal WBC count. Dry tap was observed in 84% of the cases where hairy cells constituted 16-97% of non-erythroid nucleated cells. Tartarte resistant acid phosphate staining was positive in all the eight cases where it was done. CD5 was negative in all the cases, while CD10 was expressed in three cases (13%) and CD23 in five cases (19%).
Though pancytopenia is common, occasional patient can present with normal blood counts or leukocytosis. Few unusual findings include presence of lymphadenopathy, absence of palpable splenomegaly, and expression of CD23 and CD10 by the leukemic cells.
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