Table 1

Atypical presentations of hairy cell leukemia: a comprehensive literature review.

Ref.	Age (yr)/sex	Presentation	Hairy cells			Immunophenotyping (FC/IHC)	Therapy; outcome	Molecular studies

			PBS	BMA	BMBx/tissue Bx
[3]	52/M	Pancytopenia (? aplastic); no spleen	-/+	Scant, -/+	Hypoplastic atypical lymphoid nodule	CD200/CD11c/CD25/CD20/kappa: +	Cladribine; CR	BRAF+
[3]	56/M	Pancytopenia (? aplastic)no spleen	-	-	Hypoplastic focal fried egg	CD20/annexin A1; +	Cladribine; CR	Not tested
[3]	48/M	Lymphocytosis; massive spleen, multiple LNs	-/+	-/+	LPN; fried egg	CD200/CD11c/CD25/CD20/kappa; +	Supportive	Not tested
[3]	55/F	Pancytopenia (? aplastic);no spleen	-	?/+	Hypoplastic, atypical lymphoid nodule	CD200/CD11c/CD25/CD20/kappa; +	Cladribine; CR	BRAF+
[4]	43/F	Arthalgia pancytopenia, no spleen	-/+	-/+	Fried egg	CD200/CD11c/CD25/CD20/FMC7/HLA-DR; +	Cladribine; not described	Not tested
[5]	68/M	Lesion in skull base, bones, pleura; low platelet; no spleen mediastinal LN	-	-/+	LPN (tissue and BM)	Cyclin D1+, CD 19/CD 20; +, CD5/CD23/CD10; -, CD 11c/25/103; +	Cladribine; rituximab; CR	t (11; 14); - BRAF-
[6]	55/M	Lytic to sclerotic bone lesion; rt femur, no spleen	-	-	LPN (bone biopsy) fried egg BM; no abnormality	TRAP/annexin A1/CD20/CD11c; +, FC: no abnormality	RT+ Cladribine, CR	BRAF+
[8]	44/F	Breast lesion; no spleen	-/+	Dry tap	<10% atypical lymphoid cells, MF 2	Breast: CD20/PAX5/Cyclin D1 (variable+)/annexin A1/TRAP;+ CD25; weak+ BM-FC: + (kappa)	Not described	t (11; 14); - BRAF+
[9]	69/M	Pancytopenia(? aplastic) no spleen	-	-/+	Hypoplastic, atypical lymphoid nodule, fried egg	IHC: DBA44/annexin A1; + FC: hairy cell leukemia	Cladribine; CR	Not tested
[9]	50/M	Weakness, weight loss, bicytopenia; no spleen	-/+	-/+	LPN; fried egg	TRAP (PBS); + FC; hairy cell leukemia	Cladribine; CR	Not tested
[9]	43/M	Recurrent URTI, low platelet; no spleen	-/+	-/+ (TRAP +ve)	LPN; fried egg	CD200/CD11c/CD25/CD20/kappa; +	Supportive	Not tested
[9]	67/M	Pancytopenia (? aplastic/MDS) no spleen	10%	-/+ (TRAP +ve)	Hypoplastic; atypical lymphoid nodule,fried egg, MF 1 to 2	PB-FC: CD 19/CD 11c/CD 25/CD 103; +	Cladribine; CR	Not tested
[10]	54/M	LS epidural mass with radiculopathy, multifocal bone lesions; no spleen	-	Dry tap	LPN fried egg (tissue Bx+BM)	Bx: CD20/TRAP/CD25; + BM-FC: CD19/CD11c/CD103/CD25; +	Cladribine; CR	Not tested
Present case	63/M	Breathlessness; mediastinal LN, leukopenia; no spleen	-/+	-/+	LPN; Fried egg	IHC; CD 20/CD 10/CD 11c; +, FC; CD200/CD19/CD11c/CD103/CD 10/CD25; +CD 23; dim to moderate+	Cladribine, CR	Not tested

Abbreviations: +, positive; -, negative; -/+, occasional to very few; ?/+, suspicious; BMA, bone marrow aspiration; BMBx, bone marrow trephine biopsy; BRAF, BRAF V600E mutation analysis; CD200, bright positivity in flow cytometry; CR, complete response; F, female; FC, flow cytometry; IHC, immunohistochemistry; LN, lymph nodes; LPN, low grade lymphoproliferative neoplasm; LS, lumbosacral; M, male; MDS, myelodysplastic syndrome; MF, myelofibrosis grade as per WHO; PB, peripheral blood; PBS, peripheral blood smear; RT, radiotherapy; URTI, upper respiratory tract infection.

Blood Res 2020;55:123~127 https://doi.org/10.5045/br.2020.2020069