OBJECTIVE: Hairy cell leukemia (HCL) is a well-known lymphoproliferative disease with very effective treatment approaches primarily relying on purine analogues. However, these treatments are associated with profound and prolonged immunosuppression. Merkel cell carcinoma (MCC) is a rare and extremely aggressive skin tumor with an increased incidence in immunocompromised patients.
METHODS: We report a case of a patient with HCL who was diagnosed with MCC, while in remission following retreatment with pentostatin, which induced a profound decrease in CD4 (+) T-cells.
CONCLUSIONS: Our case provides further evidence supporting the hypothesis of a significant association between immunosuppression and MCC pathogenesis.

Hairy cell leukemia (HCL) is a rare lymphoproliferative disorder classically presenting with cytopenia and recurrent infections but atypical manifestations such as bone lesions, skin lesions and effusion have been described. We report here an unusual meningeal localization in a 33 years old man who presented with headache, hand paresthesia and visual symptoms. Brain magnetic resonance imaging revealed an occipital meningeal lesion. Diagnostic explorations led to the diagnosis of classical HCL with meningeal localization. After treatment by cladribine and rituximab the patient rapidly improved and is still in complete remission 12 months after end of treatment. The literature review identified 9 other cases of HCL with central nervous system localization (CNS) presenting with brain parenchyma and/or meninges localization. Four out of 9 patients presented with hyperleukocytosis. Most patients experienced good responses with various treatments. Cladribine alone or with rituximab led to complete responses similar to our patient. In our patient, molecular biology revealed KLF2 mutations, which implication in the atypical localization could be suspected but would need dedicated studies. In conclusion, CNS localizations of HCL are rare but can be observed and treatment with cladribine alone or with rituximab appears as an effective strategy.

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.
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.
We report a case of a rare clinical presentation of SCNSI in HCL with literature review.

Hairy cell leukemia (HCL) represents a rare B-cell malignancy with 2% of all leukemias and should be differentiated from HCL-like conditions, including HCL-variant (HCL-V) and splenic diffuse red pulp lymphoma (SDRPL). HCL gets its name from the short, thin projections that look like hair on its cells. It is associated with a specific immunophenotypic profile, cytopenia, and splenomegaly. Spontaneous splenic rupture can be a symptom of hematological malignancy such as HCL and is a life-threatening acute emergency. Here, we present a case of a 37-year-old man who presented to the hospital with signs of acute peritonitis and acute anemia and was found to have atraumatic splenic rupture secondary to splenomegaly. He underwent emergent angiography, where the bleeding splenic vessel was identified, and the patient was successfully treated with embolization. Immunophenotypic profile revealed that B-cells were positive for CD11c, CD103, CD25, and CD5, for which he received five days of cladribine and achieved complete clinical remission.

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.

Hairy cell leukemia (HCL) is a rare malignancy that primarily affects the bone marrow, peripheral blood, and spleen. The most common presenting features of HCL are splenomegaly or cytopenias causing fatigue, infections, or hemorrhagic manifestations. Symptoms involving the soft tissue or bone are rare. HCL can rarely present with immune-mediated polyarthritis. This presentation can be confused for other pathological entities, such as Felty\'s syndrome, and can be differentiated from this with bone marrow biopsy. This case report looks into a rare presentation of HCL in which transient polyarthritis of the knees was the presenting symptom.

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.

Hairy cell leukemia (HCL) is a rare mature B-cell lymphoproliferative disorder and most often presents as classic hairy cell leukemia. This entity is characterized by an indolent course and the presence of the BRAF V600E mutation. We report the case of an 80-year-old man with a history of classical hairy cell leukemia who presented with fatigue, dizziness, shortness of breath, blurring of vision, and headache. His initial diagnosis was 9 years prior, and he received treatments with cladribine, pentostatin, and rituximab. The workup showed an elevated white blood cell count with atypical lymphocytes, anemia, and thrombocytopenia. A peripheral blood smear confirmed HCL relapse, and a magnetic resonance imaging (MRI) of the brain showed diffuse, nonenhancing masses in the supratentorial and infratentorial regions of the brain. He was initiated on treatment with vemurafenib, with improvements in his white blood cell count and a recovery of his platelet count and hemoglobin. A repeat MRI of the brain after 3 months showed complete resolution of the lesions. Vemurafenib was discontinued after 6 months, with bone marrow biopsy showing no evidence of residual hairy cell leukemia. There have only been limited reports of HCL involvement in the central nervous system in the literature. Due to the rarity of the condition, it is not clear which treatments can be effective for intracranial disease control. Our report shows the successful use of vemurafenib, resulting in complete remission of relapsed HCL with CNS involvement.

Hairy cell leukemia (HCL) is a rare disease of mature B-cell neoplasms. Its name comes from the hair-like strands surrounding the cytoplasm of the cells, which are observed on peripheral blood or bone marrow smears. Leukemic cells mainly involve the spleen, peripheral blood, and bone marrow. The classical immunophenotyping of HCL includes overexpression of the B-cell surface antigens such as CD19, CD20, and CD22 and co-expression of CD25, CD103, CD11c, and CD123. Other markers including CD5, CD10, and CD38 are usually negative, in which CD38 is considered a poor prognostic factor. Herein, we report a case of HCL with atypical morphology and abnormal expression of both CD38 and CD10.

A case of concomitant hairy cell leukemia (HCL) and chronic lymphocytic leukemia (CLL) in a 50- year-old man was reported. Flow cytometry and droplet digital PCR (ddPCR) were used to detect the B-Raf proto-oncogene (BRAF) V600E mutation. The HCL population was the predominant component. The patient was first treated with cladribine and then with rituximab and achieved HCL partial remission. Importantly, the high sensitivity of our flow cytometric approach allowed the detection of a small population \"P3,\" in addition to the typical HCL and CLL clones. The P3 clone changed over time, from an HCL-like to a CLL-like immunophenotype. This case is added to the few other cases of synchronous HCL and CLL already reported in the literature and underlines the importance of analyzing chronic lymphoproliferative disorders by highly sensitive diagnostic techniques, like the multicolor flow cytometry and ddPCR, to evaluate the possible association between HCL and CLL at diagnosis.