Abstract
Essential thrombocythemia (ET) is an extremely rare myeloproliferative neoplasm (MPN) which is characterized by persistent, non-reactive thrombocytosis (> 450 X 109/L) and an increased risk for thrombotic events.These patients have an increased risk of both hematological and non-hematological malignancies however the risk of breast cancer in MPN is not increased in comparison to general population. We hereby present an extremely unusual case of ET in a 38 years old female (diagnosed with breast cancer), which came to light on routine pre-surgical work up of the patient.Diagnosis was confirmed on bone marrow aspiration clubbed with JAK 2 mutation. Patients with ET have an increased risk of thrombosis thereby it becomes challenging for the clinician to operate on a coexistent solid tumor. On extensive literary search, there was a single case of coexistent Breast Cancer and Essential Thrombocythemia and we are reporting second such coexistence to the best of our knowledge. Bangladesh Journal of Medical Science Vol. 21 No. 02 April’22 Page : 463-466
Highlights
Myeloproliferative neoplasms (MPN) are a clonal hematopoietic stem cell disorder comprising of ABL positive Chronic myeloid leukemia, Polychythemiavera, Essential thrombocythemia (ET) and primary myelofibrosis
Multiple sub-centimeter size bilateral axillary lymph nodes with no significant FDG uptake were seen.Patient was diagnosed as a case of Essential Thrombocythemia in the setting of breast cancer
WHO diagnostic criteria for ET include: (1) Platelet count > 450 X 106/cumm, (2) bone marrow (BM) biopsy showing proliferation mainly of the megakaryocytic lineage with increased numbers of enlarged, mature megakaryocytes with hyperlobulated nuclei, (3) Presence of JAK2, CALR or MPL mutation (4) Not meeting WHO criteria for BCR-ABL1 + Chronic Myeloid Leukemia (CML), Polycythemia Vera (PV), Primary Myelofibrosis (PMF), Myelodysplastic syndrome (MDS) or other myeloid neoplasms with absence of any evidence for reactive thrombocytosis.4In the present case, there was isolated persistent thrombocytosis with megakaryocytic hyperplasia and enlarged hyperlobulated forms, thereby excluding other MPNs
Summary
Myeloproliferative neoplasms (MPN) are a clonal hematopoietic stem cell disorder comprising of ABL positive Chronic myeloid leukemia, Polychythemiavera, Essential thrombocythemia (ET) and primary myelofibrosis. ET is an extremely rare disease and is characterized by persistent, nonreactive thrombocytosis (> 450 X 109/L) with an increased risk for thrombotic/bleeding events.1Patients of Essential Thrombocythemia have an increased risk of developingboth hematological and non-hematologicalmalignancies there is not much increased risk of breast cancer in such. A 38 years old female came to Surgical out-patient department with lump, left breast. The lump was present in upper outer quadrant, firm in consistency and measuring 3 X 2 cms. Multiple sub-centimeter size bilateral axillary lymph nodes with no significant FDG uptake were seen.Patient was diagnosed as a case of Essential Thrombocythemia in the setting of breast cancer. The patient received adjuvant chemotherapy for breast cancer- anthracycline and cyclophosphamide for four cycles followed by taxane. Patient was started on aromatase inhibitor instead of selective estrogen receptor modulator in view of the increased risk of thromboembolic phenomenon
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