BACKGROUND: Splenic B-cell lymphoma/leukemia with prominent nucleoli (SBLPN) aka hairy cell leukemia variant (HCL-v) is a rare B-cell chronic lymphoproliferative disorder. The main diagnostic challenge is to differentiate SBLPN from Classical hairy cell leukemia (HCL-c), as the former faces inferior responses to therapies and a poor prognosis.
OBJECTIVE: The aim is to discuss the clinic-hematological and immunophenotyping findings of three cases of SBLPN.
METHODS: This is a retrospective observational study.
METHODS: From the year 2011 to 2021, flow cytometry of all the cases with HCL diagnosis was reviewed, and three cases with negative or dim CD25 and hematological presentation matching with SBLPN were picked up.
METHODS: Descriptive statistics is used.
RESULTS: All the cases were male. The age ranges from 43 to 64 years. Median hemoglobin concentration, total leucocyte count, and platelet count were 8.6 g/dL, 6.9 × 109/L, and 53 × 109/L, respectively. The atypical cells were medium to large. All three showed prominent nucleoli. Bone marrow biopsies showed an interstitial pattern of infiltration in all the cases. The hairy cells were positive for CD20, CD11c, and CD103. CD25 was dim positive in one case. Annexin A1 was negative in all three cases. BRAF V600E mutation analysis was done in one case and turned out negative for the mutation.
CONCLUSIONS: SBLPN is a rare entity, usually on-flow cytometry CD25 negative. However, in dim CD25-positive cases, BRAFV600E mutational analysis helps in discerning SBLPN diagnosis and differentiating it from HCL-c.

BACKGROUND: Hairy cell leukemia (HCL) is an indolent B-cell lymphoma characterized by a specific genetic mutation, BRAF V600E, which affects the specific morphology and oncogenesis. For HCL, few reports regarding secondary central nervous system involvement (SCNSI) are available. Herein, we present the case of an 80-year-old woman who had a relapse of HCL with SCNSI.
METHODS: The diagnosis of HCL was made in June 2015 after identifying BRAF V600E proteins by immunohistochemical analysis, and the disease was then controlled for 6 years by employing chemoimmunotherapy. In February 2021, the patient was admitted with neurological symptoms such as dizziness. Magnetic resonance imaging of the brain showed abnormal enhancement in the cerebrum, and cerebrospinal fluid analysis revealed neoplastic cells without transformation into large cells. Thus, the patient was diagnosed as having SCNSI in HCL.
CONCLUSIONS: We report a case of a rare clinical presentation of SCNSI in HCL with literature review.

Hairy Cell Leukemia is an infrequent leukemia that can be recognized both microscopically and flow cytometrically once the patient develops symptoms. We present a case where early diagnosis was achieved using flow cytometry long before the patient became symptomatic. This was achieved by focusing on a small percentage (0.9%) of total leukocytes that exhibited a higher side scatter and brighter CD19/CD20 than the remaining lymphocytes. A bone marrow aspirate three weeks later confirmed the presence of malignant B-cells. Shortly after, the patient presented splenomegaly and complained of fatigue.

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The association between pesticide exposure and non-Hodgkin lymphoma (NHL) including hairy cell leukemia (HCL) was analyzed in a pooled study of three case-control studies. Results on exposure to pesticides were based on 1,425 cases and 2,157 controls participating in the studies. Exposures were assessed by self-administered questionnaires completed as needed by phone. In the pooled univariate analyses adjusted by age, gender and year of diagnosis, exposure to herbicides of the phenoxyacetic acid type yielded statistically significant increased risk with odds ratio (OR) = 1.9, 95% confidence interval CI) = 1.4-2.5. The herbicide glyphosate gave OR = 2.2, 95% CI = 1.3-3.8. Impregnating agents increased the risk. No clear dose-response effect was seen. OR was highest in the >10-20 years latency group for herbicides and impregnating agents. In the multivariate analysis including main pesticide groups, statistically significant increased risk was found for herbicides, OR = 1.6, 95% CI = 1.2-2.1 and impregnating agents with OR = 1.4, 95% CI = 1.1-1.8. This analysis confirmed an association between NHL including HCL and exposure to certain herbicides.

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The detailed clinical course of coronavirus disease 2019 (COVID-19) in patients with hairy cell leukemia (HCL) is rarely reported. We report the first case of HCL diagnosed with prolonged pancytopenia after COVID-19 infection in Japan. We describe the case of a 56-year-old man who was diagnosed with COVID-19. Computed tomography revealed ground-glass opacities in the bilateral lung lobes as well as splenomegaly. Remdesivir and dexamethasone were administered for the treatment of COVID-19. Since the pancytopenia persisted, bone marrow examination was performed, and he was diagnosed with HCL. Although pancytopenia can occur with COVID-19 alone, clinicians should be alerted regarding the presence of hematologic malignancies in patients in whom pancytopenia persists after COVID-19 treatment or in those with splenomegaly. Further, the condition of all previously reported patients with COVID-19 associated with HCL was severe enough to require mechanical ventilation. This is the first case in which the disease was not severe. The interleukin-6 (IL-6) level was lower in this case than in previous cases, suggesting that racial differences in IL-6 production may have contributed to COVID-19 severity.

Mature B-cell lymphoproliferation with hairy lymphocytes include Marginal Zone Splenic Lymphoma (SMZL), Hairy Cell Leukemia (HCL), Splenic Diffuse Red Pulp Lymphoma (SDRPL), and Variant Hairy Cell Leukemia (HCL-v), the two latter being provisional entities that appeared in the 2008 WHO classification. We report the case of a 75-year-old man who benefited from a diagnostic re-evaluation of his SMZL. The good clinical evolution, the flow cytometry investigation (HCL score < 3, SDRPL score > 3, strong CD180 and CD200/CD180 ratio < 0.5) and the histological assessment favored a SDRPL. This entity did not exist at the time of the diagnosis in 2006. The differential diagnosis between these diseases sometimes remains uneasy. Here are mentioned some practical clues to assess the diagnosis.
Les syndromes lymphoprolifératifs B matures avec des lymphocytes d’aspect « chevelus » comprennent le lymphome splénique de la zone marginale splénique (SMZL), la leucémie à tricholeucocytes (HCL), le lymphome diffus de la pulpe rouge (SDRPL) et la leucémie à tricholeucocytes variante (HCL-v), ces deux dernières étant des entités provisoires apparues dans la classification OMS 2008. Nous rapportons le cas d’un homme de 75 ans qui a bénéficié d’une réévaluation diagnostique de son SMZL. En effet, la bonne évolution clinique, les données des explorations par cytométrie en flux (score HCL < score SDRPL > 3, CD180 fort et ratio CD200/CD180 < 0,5) et les données anatomopathologiques ont conclu à un SDRPL. Cette entité n’existait pas lors du diagnostic en 2006. Le diagnostic différentiel entre ces différentes pathologies n’étant pas toujours aisé, nous tenterons de donner quelques pistes pratiques pour conduire au diagnostic précis.

BACKGROUND: Hairy cell leukemia (HCL) is a rare indolent lymphoproliferative disease with an accumulation of mature B lymphocytes with fine reticular chromatin and cytoplasm with typical hairy-like cytoplasmic projections. Rarely, hairy cell leukemia manifests as a lung infiltration. The differential diagnosis between infection and malignant involvement with hairy cell leukemia is often challenging in such situations.
RESULTS: We present a 53-year-old female with an uncommon pulmonary involvement with hairy cell leukemia. In addition, we discuss the complicated differential diagnosis of pulmonary disease in patients with hairy cell leukemia and the treatment approach to these patients.
CONCLUSIONS: This case report describes the successful therapy management of a patient with pulmonary involvement by hairy cell leukemia. Therapy with interferon-alfa and cladribine resulted in long-term remission of the underlying disease.

暂无摘要。