Contributions
Abstract: PB1796
Type: Publication Only
Background
Hairy cell leukemia (HCL) is a rare chronic lymphoproliferative disease, comprising 2% of all adult leukemia cases. With current treatment, including purine nucleoside analogs, the prognosis of HCL is favorable, with some reports of 12-y overall survival rate of 87%. Usually HCL presents with cytopenias, enlarged spleen, massive lymphadenopathy and secondary infections, as tumor cells predominantly affects bone marrow and spleen, but rarely it can be presented with atypical involvement of bone, liver, skin, neurologic, or vascular tissue. CNS involvement, reported in only few cases, is extremely rare, most neurological signs and symptoms are usually related with infections.
Aims
To present diagnostics, clinical course and treatment outcome of our patient diagnosed with variant HCL (vHCL) and very rare leukemia CNS involvement.
Methods
Case report of a rare vHCL with CNS involvement.
Results
Our female patient, aged 68, was initially admitted as a high suspicion for subacute meninogencephalitis, in the Clinic for Infectious Disease, Clinical Centre of Serbia. She presented with persistent high fever and neurological/ neurological related symptoms (generalized convulsions, aphasia, confusion, nausea and vomiting) of which some (confusion, nausea) appeared 3 months earlier. Magnetic resonance (MR) of the brain was highly suspicious for bilateral frontal lobes empyema. Serological and cerebrospinal fluid (CFS) analyses for WNV, Wright, HSV1 and 2, Toxoplasma Gondii, Rubeolla, CMV and B. Burdgoferii, as well as for TBC and Cryptococcus were all negative. Lumbal puncture test for malignant cells was negative, but revealed a large number of small and active lymphocytes. Blood count showed bicytopenia (hemoglobin 9.7g/dl, WBC 1.7x109/l). Laboratory analyses showed high C-reactive protein and ESR. Radiography findings (ultrasound and CT) revealed no enlarged spleen or lymph nodes. However, CSF Flow cytometric immunophenotyping (FCI) analysis revealed significant (56%) presence of B-Non Hodgkin Lymphoma (B-NHL) cell population, CD19+highCD20+mediumCD103+lowCD45+high/SSClow, confirming malignant CNS involvement. Patient was initially treated with antiepileptics, broad spectrum antibiotics, antituberculosis and antifungal therapy. After subsequent admission in our Clinic, hematological diagnostics was performed. Peripheral blood smear was indicative for vHCL, showing atypical TRAP+. FCI analyses of the peripheral blood lymphocytes and of bone marrow revealed presence of atypical monoclonal mature B cells in peripheral blood (170cells/µl, 10% of WBC) and in bone marrow respectively (24% of nuclei cells), implying presence of B-NHL, CD5-, CD10-, CD103+ in leukemic phase. CLL score was 3. Aspiration biopsy, as well as bone marrow biopsy, showed significant (up to 90%) presence of small cell lymphoid infiltrates. Following these findings and regarding absence of lymphoid tissue tumor burden, lack of enlarged spleen, and aggressive clinical presentation, we diagnosed our patient with vHCL and extremly rare leukemia involvement of CNS. Patient was treated accordingly with Cladribine, one cycle, plus intrathecal terapy with Metotrexate. Post treatment analyses showed good partial responce with complete regresssion of neurological signs and symptoms and normal posttherapeutical brain MR finding.
Conclusion
We presented a case of an extremely rare leukemia involvement of CNS with vHCL cells. Leukemia cells were found in CSF and final regression of neurological signs and symptoms was registered after Cladribine treatment.
Session topic: 21. Aggressive Non-Hodgkin lymphoma - Clinical
Keyword(s): CNS, Hairy cell leukemia
Abstract: PB1796
Type: Publication Only
Background
Hairy cell leukemia (HCL) is a rare chronic lymphoproliferative disease, comprising 2% of all adult leukemia cases. With current treatment, including purine nucleoside analogs, the prognosis of HCL is favorable, with some reports of 12-y overall survival rate of 87%. Usually HCL presents with cytopenias, enlarged spleen, massive lymphadenopathy and secondary infections, as tumor cells predominantly affects bone marrow and spleen, but rarely it can be presented with atypical involvement of bone, liver, skin, neurologic, or vascular tissue. CNS involvement, reported in only few cases, is extremely rare, most neurological signs and symptoms are usually related with infections.
Aims
To present diagnostics, clinical course and treatment outcome of our patient diagnosed with variant HCL (vHCL) and very rare leukemia CNS involvement.
Methods
Case report of a rare vHCL with CNS involvement.
Results
Our female patient, aged 68, was initially admitted as a high suspicion for subacute meninogencephalitis, in the Clinic for Infectious Disease, Clinical Centre of Serbia. She presented with persistent high fever and neurological/ neurological related symptoms (generalized convulsions, aphasia, confusion, nausea and vomiting) of which some (confusion, nausea) appeared 3 months earlier. Magnetic resonance (MR) of the brain was highly suspicious for bilateral frontal lobes empyema. Serological and cerebrospinal fluid (CFS) analyses for WNV, Wright, HSV1 and 2, Toxoplasma Gondii, Rubeolla, CMV and B. Burdgoferii, as well as for TBC and Cryptococcus were all negative. Lumbal puncture test for malignant cells was negative, but revealed a large number of small and active lymphocytes. Blood count showed bicytopenia (hemoglobin 9.7g/dl, WBC 1.7x109/l). Laboratory analyses showed high C-reactive protein and ESR. Radiography findings (ultrasound and CT) revealed no enlarged spleen or lymph nodes. However, CSF Flow cytometric immunophenotyping (FCI) analysis revealed significant (56%) presence of B-Non Hodgkin Lymphoma (B-NHL) cell population, CD19+highCD20+mediumCD103+lowCD45+high/SSClow, confirming malignant CNS involvement. Patient was initially treated with antiepileptics, broad spectrum antibiotics, antituberculosis and antifungal therapy. After subsequent admission in our Clinic, hematological diagnostics was performed. Peripheral blood smear was indicative for vHCL, showing atypical TRAP+. FCI analyses of the peripheral blood lymphocytes and of bone marrow revealed presence of atypical monoclonal mature B cells in peripheral blood (170cells/µl, 10% of WBC) and in bone marrow respectively (24% of nuclei cells), implying presence of B-NHL, CD5-, CD10-, CD103+ in leukemic phase. CLL score was 3. Aspiration biopsy, as well as bone marrow biopsy, showed significant (up to 90%) presence of small cell lymphoid infiltrates. Following these findings and regarding absence of lymphoid tissue tumor burden, lack of enlarged spleen, and aggressive clinical presentation, we diagnosed our patient with vHCL and extremly rare leukemia involvement of CNS. Patient was treated accordingly with Cladribine, one cycle, plus intrathecal terapy with Metotrexate. Post treatment analyses showed good partial responce with complete regresssion of neurological signs and symptoms and normal posttherapeutical brain MR finding.
Conclusion
We presented a case of an extremely rare leukemia involvement of CNS with vHCL cells. Leukemia cells were found in CSF and final regression of neurological signs and symptoms was registered after Cladribine treatment.
Session topic: 21. Aggressive Non-Hodgkin lymphoma - Clinical
Keyword(s): CNS, Hairy cell leukemia