Role of blood and bone marrow examination in the diagnosis of mature lymphoid neoplasms in patients presenting with isolated splenomegaly

Figures & data

Table 1. Demographic and hematological profile of patients with prominent splenomegaly without lymphadenopathy

Number of patients	75
Age mean [range] in years	53 (17–78)
Male female ratio	1.7:1
Number of patients (%) with splenomegaly
Mild	8 (10.6%)
Moderate	23 (30.6%)
Massive	44 (58.6%)
Number of patients with hepatomegaly	43 (57%)
Number of patients with anemia (with or without thrombocytopenia/leukopenia)	39 (52%)
Number of patients with bicytopenia	34 (45.3%)
Number of patients with pancytopenia	27 (36%)
Absolute lymphocyte count mean (range)	14.013 (0.1–310.6) × 10^9/L
Number of patients with absolute lymphocytosis (>4 × 10^9/L)	25 (33%)

Figure 1. The categories of mature lymphoid neoplasms, their frequencies and the diagnostic method utilized for their immunophenotyping in patients with prominent splenomegaly without lymphadenopathy.

Figure 2. The morphological features of lymphoid cells in peripheral blood and/ or bone marrow aspirate (100x; May Grunwald Giemsa stain) (A) Hairy cells of HCL. (B) SMZL showing monocytoid lymphocytes. Short polar villi seen in one of them (C) Villous lymphocyte of SMZL (D) CLL showing small lymphocytes along with occasional prolymphocytes (E) B-cell PLL showing predominantly prolymphocytes (F) MCL showing lymphoid cells with irregular cleaved nucleus (G) Intravascular large B cell lymphoma showing blastoid appearing lymphoid cells (H) γδ HSTL showing medium to large sized lymphoid cells (I) CLPD NK cells showing large granular lymphocytes.

Figure 3. The morphology and salient immunohistochemistry findings of mature lymphoid neoplasms in bone marrow trephine biopsy. HCL showing typical ‘fried egg’ appearance (A) [Haematoxylin and eosin (H&E), 40x] and cytoplasmic positivity for CD72 (DBA.44) (B) [IHC, 40x]. MCL showing diffuse infiltration of bone marrow (C) [H&E, 40x] with nuclear positivity for cyclin D1 (D) [IHC, 40x]. SMZL showing nodular lymphoid infiltrate (E) [H&E, 10x] with intrasinusoidal and interstitial infiltrate highlighted by CD20 (F) [IHC, 20x]. DLBCL showing diffuse infiltration by large atypical lymphoid cells (G) [H&E, 40x] which are positive for CD20 (H) [IHC, 40x]. Intravascular large B cell lymphoma showing scattered atypical large cells (I) (H&E, 40x), the intrasinusoidal nature of which is highlighted by CD20 (J) [IHC, 40x]. Diffuse infiltrate of atypical large lymphoid cells with prominent eosinophilic nucleolus (K) [H&E, 40x], which are CD20⁺ CD5⁺ CD10⁻ (not shown) and Cyclin D1⁻ (L) [IHC, 40x] raising a differential diagnosis of cyclin D1 negative blastoid MCL and CD5⁺ DLBCL.

Table 2. Summary of clinical and hematological findings of mature lymphoid neoplasms diagnosed in patients with prominent splenomegaly without lymphadenopathy

Category	Age median ± SD (range) in years	Male: Female	Patients with splenomegaly			Patients with lymphocytosis (%)	Patients with pancytopenia (%)
			Mild (%)	Moderate (%)	Massive (%)
Mature B cell neoplasms
HCL (n = 24)	46 ± 13.4 (28–75)	3.8:1	5 (20.8)	8 (33.3)	11 (45.8)	3 (12.5)	17 (70.8)
SMZL (n = 18)	66 ± 11 (35–75)	1:1.3	1 (5.6)	4 (22.2)	13 (72.2)	8 (44.4)	4 (22.2)
DLBCL (n = 6)	48.5 ± 20 (17–68)	1:2	1 (16.7)	3 (50)	2 (33.3)	0	3 (50)
CLL^# (n = 4)	70.5 ± 17 (41–78)	3:1	0	1 (25)	3 (75)	4 (100)	0
SLL (n = 2)	55, 56	2 males	0	0	2 (100)	0	1
HCL v (n = 2)	72, 76	2 males	0	2 (100)	0	1 (50)	1 (50)
MCL (n = 2)	50, 55	2 males	0	1 (50)	1 (50)	2 (100)	0
PLL (n = 1)	70	Male	0	0	1 (100)	1 (100)	0
FL (n = 1)	65	Female	0	0	1 (100)	1 (100)	0
B-NHL, NOS (n = 6) – see text
Mature T/NK cell neoplasms
HSTL (n = 5)	29 ± 3 (25–33)	1:1.5	0	1 (20)	4 (80)	2 (40)	0
CLPD NK cells (n = 1)	41	Male	0	0	1 (100)	0	1 (100)
T-NHL, NOS (n = 3) – see text

^#Includes a case of CLL/PLL. SD – standard deviation.

Figure 4. The distribution of mature lymphoid neoplasms in patients presenting with massive (A), moderate (B), and mild (C) splenomegaly.