De novo chronic lymphocytic leukemia/prolymphocytic leukemia or B-cell prolymphocytic leukemia? The importance of integrating clinico-morphological and immunophenotypic findings in distinguishing chronic lymphoproliferative diseases with circulating phase

Chowdhury, Zachariah; Khonglah, Yookarin; Sarma, Susmita; Kalita, Pranjal

doi:10.4322/acr.2020.196

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Sumário

Clinical Case Report • Autops. Case Rep. 11 • 2021 • https://doi.org/10.4322/acr.2020.196 copy

De novo chronic lymphocytic leukemia/prolymphocytic leukemia or B-cell prolymphocytic leukemia? The importance of integrating clinico-morphological and immunophenotypic findings in distinguishing chronic lymphoproliferative diseases with circulating phase

Authorship SCIMAGO INSTITUTIONS RANKINGS

ABSTRACT

B-cell prolymphocytic leukemia (B-PLL) is an extremely rare disease, accounting for approximately 1% of the lymphocytic leukemias. B-PLL generally occurs in older people. It is characterized by the presence of more than 55% prolymphocytes in the peripheral blood (PB), no or minimal lymphadenopathy, massive splenomegaly, and very high white blood cell counts. The prognosis of B-PLL patients is generally poor, with a median survival of 3 years, although a subset of patients may show a prolonged survival. Herein, we report a case of a 70-year-old male with weakness, generalized lymphadenopathy, and moderate splenomegaly at the initial presentation. Hematologic examination revealed lymphocytic leukocytosis, favoring a chronic lymphoproliferative disorder (CLPD). The key to decoding the precise CLPD was a combination of the clinical profile, morphologic findings on the peripheral blood and the bone marrow, immunophenotypic analysis, and cytogenetic study. The best diagnosis proffered was a de novo chronic lymphocytic leukemia/prolymphocytic leukemia. There was no prior history of lymphoproliferative disorder or lymphocytic leukocytosis. Discriminating this entity from other lymphoproliferative disorders is crucial as the treatment and prognosis are varied compared to the other lymphoproliferative disorders. The diagnostic conundrum encountered and the incredible utility of ancillary studies in such a scenario are highlighted in this study.

Keywords
Immunophenotyping; Leukemia, Lymphoid; Lymphadenopathy: Rare Diseases

Parameters	CLL/PLL		B-PLL	CLL	HCL	HCL-V	SMZL	MCL	FL
Generalised Lymphadenopathy	Present		Absent	Present	Uncommon	Uncommon	Uncommon	Present	Present
Splenomegaly	Moderate to massive		Massive	Mild to moderate	Massive	Moderate to massive	Moderate to massive	Mild to moderate	Mild to moderate
Lymphocyte count	Variably increased		High, > 100 x 10⁹/L	Variably increased	Normal or low, Pancytopenia with monocytopenia	Modest, 20-40 x 10⁹/L; No monocytopenia	Usually normal or low level increase	Usually < 50 x 10⁹/L	Normal or low level increase
Lymphoid cell morphology	Mixture of small CLL lymphocytes and Prolymphocytes (11-55%)		Predominantly prolymphocytes (> 55%), usually > 90%	Predominantly small lymphocytes; prolymphocytes < 11%	Lack nucleoli, nuclei indented, ‘hairy’ cytoplasmic projections	Prominent central nucleolus, irregular nuclear contours, ‘hairy’ cytoplasmic projections	Short polar villi, basophilic cytoplasm	Heterogeneous, larger indented nuclei	Inconspicuous nucleoli, small irregular cleaved nuclei resembling centrocyte
Immunophenotype
B cell antigens (CD 19, CD 20, CD 22, CD 79a)		+ (Moderate)	+ (Strong)	+ (Dim)	+ (Strong)	+ (Strong)	+ (Moderate)	+ (Moderate)	+ (Moderate)
CD 5		+/-	- (most)	+	-	-	- (usually)	+ (most)	-
CD 23		+/-	-	+	-	-	30% + (weak)	-	+/-
Other antigens		CD 10 -	CD 10 -	CD 10 -	CD 11c +, CD 123 +, CD 25 +, CD 103 +, Annexin A1 +	CD 11c +, CD 103 +, CD 25 -, CD 123 -, Annexin A1 -	CD 103 -, CD 123 -, Annexin A1 -, Cyclin D1 -	Cyclin D1 +, SOX11 +	CD 10 +

Hospital Universitário da Universidade de São Paulo Hospital Universitário da Universidade de São Paulo, Av. Prof. Lineu Prestes, 2565 - Cidade Universitária, 05508-000 - São Paulo - SP - Brasil, (16) 3307-2068, (16) 3307-2068 - São Paulo - SP - Brazil
E-mail: autopsy.hu@gmail.com

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[1] Authors’ contributions: Zachariah Chowdhury, Yookarin Khonglah, Susmita Sarma and Pranjal Kalita equally contributed to the manuscript conception; however, Zachariah Chowdhury and Susmita Sarma were in charge of the literature research. All authors collectively proofread and approved the final version for publication.