Persistent Polyclonal B-cell Lymphocytosis With Splenomegaly

Martínez-López, Azahara; Montes‐Moreno, Santiago; Mazorra, Francisco; Miranda-Vallina, Cesar; Ulibarrena, C; Martin, Juan Luis Alfonso; Bosch, J. M.; Peri, Valeria; Burdaspal, A.; Fernandez-Alvarez, Montse; Sánchez-Verde, Lydia; Piris, Miguel Á.

doi:10.1097/pas.0b013e31828b2c77

Cited by 3 publications

(2 citation statements)

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“…Histologic sections obtained from splenectomy show infiltration of both white and red pulp, with enlargement of the marginal-zone of splenic follicles, and atypical lymphocytes in the splenic sinusoids. [11][12][13] Such morphologic findings could lead to a misdiagnosis of splenic B-cell lymphoma. 7 Taken together, the above findings support the hypothesis that B-lymphocytes in PPBL derive from the marginal zone of the spleen, circulate in the peripheral blood and migrate to the bone marrow.…”

Section: Introductionmentioning

confidence: 99%

Modifications in B-Lymphocyte Number and Phenotype in the Course of Pregnancy in a Woman with Persistent Polyclonal B-Cell Lymphocytosis: A Flow Cytometric Study

Carulli

Ciancia²,

Sammuri

et al. 2015

J Clin Exp Hematopathol

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Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare clinical condition, characterized by a persistent, generally moderate lymphocytosis, generally due to stimulation of central memory B-lymphocytes, and by a moderate increase of polyclonal IgM. In some patients, slight or moderate splenomegaly is observed. A variable percentage of circulating, bone marrow and splenic lymphocytes display an abnormal nucleus (generally bilobated) or are binucleated. The clinical course is benign in most cases and transformation into splenic B-cell lymphoma occurs in few cases. In the current paper we report the first case of pregnancy in PPBL. Our patient became pregnant 18 months after diagnosis. In the course of pregnancy, a marked down-regulation of lymphocytosis (from 6 × 10(9)/L to 2.1 × 10(9)/L) and a decrease in B-lymphocyte number was observed (from 3.6 × 10(9)/L to 1 × 10(9)/L), mainly due to a marked reduction in the percentage and absolute number of central memory B-cells. Such modifications were similar to those described in normal pregnant women. One year after the delivery of a healthy female baby, the number of total lymphocytes and B-lymphocytes showed an inverse behavior, with a new expansion of central memory B-cells. Our case shows that a normal pregnancy can occur in patients with PPBL and that pregnancy can induce marked modifications in B-lymphocyte kinetics and phenotype.

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Section: Introductionmentioning

confidence: 99%

Modifications in B-Lymphocyte Number and Phenotype in the Course of Pregnancy in a Woman with Persistent Polyclonal B-Cell Lymphocytosis: A Flow Cytometric Study

Carulli

Ciancia²,

Sammuri

et al. 2015

J Clin Exp Hematopathol

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“…Persistent leukocytosis with megalosplenia without any underlying infectious or inflammatory cause is a common manifestation among patients with myeloproliferative neoplasm (MPN), especially chronic myeloid leukemia (CML). [1][2][3] We report a rare case of myeloid neoplasm with BCR-PDGFRA rearrangement characterized by marked elevation of leukocyte count and megalosplenia, which might improve the focus on this group of diseases and potentially reduce missed diagnoses or misdiagnoses.…”

Section: Introductionmentioning

confidence: 99%

A rare cause of persistent leukocytosis with massive splenomegaly

Gao

Weng

et al. 2022

Medicine

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Rationale:Persistent leukocytosis with megalosplenia is a common manifestation among patients with myeloproliferative neoplasm (MPN), especially for chronic myeloid leukemia (CML) patients. Here, we report a rare case of myeloid neoplasm with BCR-PDGFRA rearrangement characterized by obvious elevation of leukocyte count and megalosplenia.Patient concerns:A 32-year-old man presented with persistent leukocytosis and megalosplenia.Diagnosis:This patient was characterized by increased leukocyte count and megalosplenia, and was clinically diagnosed as CML. However, the BCR/ABL fusion gene of the patient was negative, which did not support CML. Moreover, the results of the karyotype showed 46, XY, t(4;22)(q12;q11) and RT-PCR + Sanger detection showed positive PDGFA/BCR. Accordingly, the diagnosis of myeloid neoplasm with BCR-PDGFA rearrangement was confirmed.Interventions:This patient was initially received imatinib (400 mg) orally once a day, and the dosage was adjusted to 100 mg owing to suffering from grade IV bone marrow suppression.Outcomes:Hematological remission was achieved after 2 weeks, the best treatment response was achieved after 3 months, and the main molecular biological response was achieved after 12 months.Lesson:This case suggests that rare PDGFA fusion genes screening for patients comorbid with leukocytosis and megalosplenia is necessary to avoid misdiagnosis. Unlike other rearrangements of PDGFRA, the clinical manifestations of BCR-PDGFRA rearrangement are resembling CML without eosinophilia increase.

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