Unusual Presentation of Hairy Cell Leukemia: A Case Series of Four Clinically Unsuspected Cases

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.

Hairy Cell Leukemia Presenting with a Lytic Bone Lesion

“…Splenomegaly is the most common physical fi nding in HCL and is reported in 70% to 100% of cases (6). Th ere is a previous case report of 4 patients with HCL without splenomegaly who also had lytic bone lesions (7). Second, the patient did not present with pancytopenia, which is common at presentation and reported in 50% to 70% of patients with HCL (1, 2).…”

Splenectomy in Special Cases

“…Splenomegaly is present in 80-90% of patients with hairy cell leukemia [20], and splenectomy is indicated for splenomegaly and secondary symptomatic cytopenia [21] as in the case presented above. The most common classification of splenomegaly is that proposed by Hacket in 1944 [22] and places the enlarged spleen in 5 degrees: In the case of the above presented patient, the spleen has surpassed pubic symphysis, a situation not known in the literature, for which the term of extreme splenomegaly would be appropriate.…”

Atypical presentation of hairy cell leukemia: a report and comprehensive review

“…Atypical or unusual presentation in HCL (aHCL) is uncommonly reported in the literature (1990 to 2018), which may pose a significant diagnostic challenge in routine practice (Table 1) [2][3][4][5][6][7][8][9][10]. Splenohepatomegaly is conspicuously absent which, in the presence of peripheral blood cytopenia(s), resembled hypoplastic anemias.…”