Contributions
Abstract: PB1534
Type: Publication Only
Session title: Chronic myeloid leukemia - Clinical
Background
Chronic myeloid leukemia (CML) represents a common condition in the spectrum of myeloproliferative disorders. It classically exhibits leukocytosis, but rarely presents with isolated thrombocytosis. Severe thrombocytosis, with a platelet count of more than 1,000×109/L, is exceptionally rare to be found upon initial laboratory workup in newly diagnosed patients with CML. A systematic review done by Dawood Findakly et al only found 20 cases in PubMed, MEDLINE®, ScienceDirect, and Scopus for English-language articles for the period 2000-2020.
Aims
To describe a case of a patient with isolated thrombocytosis as initial manifestation of CML.
Methods
A 33 yo woman was admitted for spontaneous hematomas and extreme thrombocytosis. Laboratory was as follows: Hg 13gr/dl WBC 8400 (normal blood smear) Platelet 2 million/mm3. Factor VIII 61% V. Willebrand (VW) 70%, Ristocetin ccofactor 40%. Abdominal ultrasound showed no visceromegalies. A bone marrow biopsy was done: cellularity was 70% CD34+ cells 4% with marked hyperplasia. Hydroxyurea was initiated at an initial dose of 500mg BID and she was discharged. Essential thrombocytemia was the initial suspected diagnosis. 1 week after she was admitted again with headache and dizziness. Platelet count has risen to 3 million/mm3 despite an increase in hydroxyurea to 1gr every 8hs. Since she had neurological symptoms, she initiated platelet apheresis for 3 sessions achieving symptomatic relief and was discharged with a platelet count of 500000/mm3. Cytogenetic results were 46XX, t (9;22) and BCRABL p210 transcript by qPCR was positive . JAK2V16F was negative. CALR and MPL mutations were also negative
Results
With diagnosis of CML she iniated imatinib 400mg/daily achieving at month 6 mayor molecular response, normalization of platelet and VW factor. We present this case because of the rare incidence isolated thrombocytosis as manifestation of CML
Conclusion
This atypical CML presentation with an isolated thrombocytosis is a rare entity. Isolated thrombocytosis could lead to a misdiagnosis of essential thrombocythemia.
Keyword(s): Acquired von Willebrand syndrome, Chronic myeloid leukemia, Myeloproliferative disorder, Thrombocytosis
Abstract: PB1534
Type: Publication Only
Session title: Chronic myeloid leukemia - Clinical
Background
Chronic myeloid leukemia (CML) represents a common condition in the spectrum of myeloproliferative disorders. It classically exhibits leukocytosis, but rarely presents with isolated thrombocytosis. Severe thrombocytosis, with a platelet count of more than 1,000×109/L, is exceptionally rare to be found upon initial laboratory workup in newly diagnosed patients with CML. A systematic review done by Dawood Findakly et al only found 20 cases in PubMed, MEDLINE®, ScienceDirect, and Scopus for English-language articles for the period 2000-2020.
Aims
To describe a case of a patient with isolated thrombocytosis as initial manifestation of CML.
Methods
A 33 yo woman was admitted for spontaneous hematomas and extreme thrombocytosis. Laboratory was as follows: Hg 13gr/dl WBC 8400 (normal blood smear) Platelet 2 million/mm3. Factor VIII 61% V. Willebrand (VW) 70%, Ristocetin ccofactor 40%. Abdominal ultrasound showed no visceromegalies. A bone marrow biopsy was done: cellularity was 70% CD34+ cells 4% with marked hyperplasia. Hydroxyurea was initiated at an initial dose of 500mg BID and she was discharged. Essential thrombocytemia was the initial suspected diagnosis. 1 week after she was admitted again with headache and dizziness. Platelet count has risen to 3 million/mm3 despite an increase in hydroxyurea to 1gr every 8hs. Since she had neurological symptoms, she initiated platelet apheresis for 3 sessions achieving symptomatic relief and was discharged with a platelet count of 500000/mm3. Cytogenetic results were 46XX, t (9;22) and BCRABL p210 transcript by qPCR was positive . JAK2V16F was negative. CALR and MPL mutations were also negative
Results
With diagnosis of CML she iniated imatinib 400mg/daily achieving at month 6 mayor molecular response, normalization of platelet and VW factor. We present this case because of the rare incidence isolated thrombocytosis as manifestation of CML
Conclusion
This atypical CML presentation with an isolated thrombocytosis is a rare entity. Isolated thrombocytosis could lead to a misdiagnosis of essential thrombocythemia.
Keyword(s): Acquired von Willebrand syndrome, Chronic myeloid leukemia, Myeloproliferative disorder, Thrombocytosis