Distinctive patterns of sequential platelet counts following blunt traumatic brain injury predict outcomes.

OBJECTIVE: To determine the role of platelet counts in the context of the decision to treat patients with non-compounded, non-surgically-treated blunt traumatic brain injury (NCNS-bTBI) with anticoagulants/antiaggregants. METHODS: A retrospective analysis of 141 anticoagulants/antiaggregants-naïve patients with NCNS-bTBI. Changes in PT-INR and prolonged aPTT were examined and correlated with Marshall and Rotterdam scores, clinical and neuroradiological outcomes. RESULTS: Three groups of platelet counts were identified. Group 1 (83% of patients) had normal platelet counts (150,000-450,000 platelets/mm3) from admission to discharge. Group 2 (13%) developed transient thrombocytopenia (<150,000 platelets/mm3) 2-3 days post-trauma. Group 3 (4%) developed extreme thrombocytosis > 1,000,000/mm3 platelets 6-9 days post-trauma. Neither acute coagulopathy of trauma nor progressive hemorrhagic insults followed NCNS-bTBI. Moreover, while patients with thrombocytosis/extreme thrombocytosis presented with a worse Glasgow coma score (GCS) on admission (8.8 ± 2.9 vs. 13 ± 2, p < 0.01) and had longer hospitalization (13.5 ± 10.4 vs. 4.5 ± 2.1 days), their improvement at discharge was the highest (delta GCS, 4 ± 2.8 vs. 1.2 ± 2.1, p = 0.05). Traumatic subarachnoid hemorrhage was associated with isolated thrombocytosis and 'best improvement.' No thromboembolic or hemorrhagic complications occurred. CONCLUSION: NCNS-bTBI, thrombocytosis was correlated with better outcomes and was not associated with an increased risk for developing thromboembolism or hemorrhage, precluding the immediate need for any additional antiaggregates.

Is very high platelet count always associated with essential thrombocythemia? An unusual presentation in a child.

Myeloproliferative neoplasms are rare in childhood. They are categorized as Philadelphia chromosome-positive and Philadelphia chromosome-negative. Chronic myeloid leukemia (CML) is the most common myeloproliferative disease in which the Philadelphia chromosome is detected as a result of BCR-ABL rearrangements. In others, the most common genetic abnormality is JAK2V617F mutation. The coexistence of these 2 abnormalities in CML is unexpected, and rare cases have recently been reported in adults. We present a child who had a very high platelet count in which we found this coexistence. The clinical presentation, laboratory findings, management, and prognosis of this coexistence is challenging in such a rare condition.

A Case of JAK2V617F-Negative Myeloproliferative Neoplasm in a Young Female Presenting With Extreme Thrombocytosis.

Thrombocytosis is a commonly observed condition in clinical practice and typically results from various pathophysiological factors, such as iron deficiency, blood loss, infection, medications, rheumatologic conditions, malignancy, asplenia, post-splenectomy, or familial factors. However, extreme thrombocytosis, defined as a platelet count > 10,000 K/UL (equal or greater than a million), is a rare occurrence. In this report, we present a compelling case of severe thrombocytosis attributed to underlying chronic myelogenous leukemia (CML), further complicated by coexisting iron deficiency. It is essential to emphasize that not all instances of extreme thrombocytosis are indicative of essential thrombocythemia. Hence, maintaining a high level of suspicion for non-ET myeloproliferative neoplasms (MPNs) such as CML, as well as other underlying conditions like iron deficiency anemia, is crucial for accurate diagnosis and timely management.

Celiac Disease Masquerading as Extreme Thrombocytosis and Severe Anemia in a 52-Year-Old Female Patient: A Rare Case Presentation and Literature Review.

Celiac disease (CD) is a chronic autoimmune disorder characterized by an immune-mediated response to gluten, resulting in small intestinal mucosal damage. While gastrointestinal (GI) symptoms are commonly associated with CD, atypical presentations can pose diagnostic challenges, particularly when hematological abnormalities are the primary manifestation. We report a case of a 52-year-old female patient who presented with paraesthesia, numbness in her hands and feet, marked thinness, extreme thrombocytosis, severe anemia, and mild electrolyte imbalance. Physical examination was unremarkable, except for the notable thinness. GI symptoms were absent, and there was no family history of gastroenterological diseases. Diagnostic evaluations, including serological tests and duodenal biopsy, confirmed the diagnosis of CD with grade 4 Marsh 3C classification. This case emphasizes the significance of considering CD as a potential cause for atypical hematological manifestations, such as extreme thrombocytosis secondary to severe anemia. Prompt recognition and appropriate management, including adherence to a gluten-free diet, can lead to symptom improvement and resolution of hematological abnormalities. It is crucial for healthcare professionals to recognize and be familiar with these atypical presentations to promote early diagnosis and enhance patient outcomes.

Long-Term Molecular Remission after Treatment with Imatinib in a Chronic Myeloid Leukemia Patient with Extreme Thrombocytosis Harboring Rare e14a3 (b3a3) BCR::ABL1 Transcript: A Case Report.

An atypical BCR::ABL1 fusion gene transcript in chronic myeloid leukemia (CML) patients, even those with variant Philadelphia (Ph) chromosome translocation, is very rare. In the present study, we report a case of CML (41 years, female) with extreme thrombocytosis at onset, with the variant Ph chromosome and rare e14a3 (b3a3) BCR::ABL1 transcript. The patient was prescribed imatinib as a first-line therapy and subsequently achieved complete hematologic remission within 2 months and major molecular response (MMR) within 3 months, and the transcript was undetectable within half a year. During up to nine years of follow-up, the quantification of this rare fusion gene was consistently negative with no BCR::ABL1 kinase domain mutations. Furthermore, we collected previously reported CML cases with the e14a3 (b3a3) transcript that indicated that the e14a3 (b3a3) transcripts appeared to have a larger number of thrombocytosis and variant Ph translocations than CML in general. This subgroup of CML might have better responses and outcomes to imatinib than patients with common transcripts.

A cross-sectional retrospective study to analyze the underlying causes and clinical characteristics of children with reactive thrombocytosis at a Korean tertiary medical center.

BACKGROUND: Reactive thrombocytosis (RT) is a common condition among children, although no studies have examined the etiology or clinical characteristics of RT among Korean children. METHODS: This retrospective study evaluated children with RT at a single Korean tertiary center during a 10-year period. RESULTS: RT accounted for 13.5% of children who were admitted to the pediatric ward (4,113/30,355): mild RT, 82.7%; moderate RT, 14.1%; severe RT, 1.1%; and extreme RT, 2.1%. There was a negative correlation between platelet count and Hb level (P=0.008). There were positive correlations between platelet count and WBC (P=0.001), erythrocyte sedimentation rate (ESR) (P=0.007), and admission duration (P=0.006). The most common cause of RT was infection and the second most common was Kawasaki disease (KD). The highest proportion of lower respiratory tract infection was observed in extreme RT (P<0.001). The proportion of KD was highest in extreme RT (P<0.001) and in children aged 1-7.9 years (P<0.001). The proportion of refractory KD was highest in extreme RT (P=0.005). In cases of KD, there was a positive correlation between platelet count and fever duration (P=0.006). Non-KD autoimmune inflammation was only observed in mild/moderate RT, and its proportion was highest in children aged 8-18 years (P<0.001). CONCLUSION: In children, more severe RT was associated with lower Hb, increased WBC, ESR, and prolonged admission. With respiratory infection or KD, extreme RT was associated with more severe disease course.

Elevated telomerase activity in essential thrombocythemia with extreme thrombocytosis.

INTRODUCTION: We performed a comparative analysis of telomerase activity (TA) in patients with myeloproliferative neoplasm (MPN) and myelodysplastic syndrome (MDS). The relationships between TA and known prognostic factors were also analyzed. MATERIALS AND METHODS: A telomeric repeat amplification protocol was performed with bone marrow hematopoietic cells from 96 normal controls, 44 MPNs, and 40 MDSs. RESULT: TA (measured as molecules/reaction) showed no correlation with age in the control group (R(2)=0.0057, p=0.464). MPN showed elevated TA compared with controls (15,537.57 vs. 7775.44, p=0.020). Patients with essential thrombocythemia showed markedly elevated TA (22,688.56, p=0.030), particularly in cases with extreme thrombocytosis versus those without extreme thrombocytosis (34,522.19 vs. 9375.71, p=0.041). MDS patients showed a TA value of 7578.50. CONCLUSION: There was no association between age and TA in bone marrow hematopoietic cells. TA was elevated in MPN but borderline in MDS, probably because of differences in the nature of the diseases. Elevated TA in patients with essential thrombocythemia, especially those with extreme thrombocytosis, suggests that an anti-telomerase strategy could be beneficial in the prevention of thrombotic complications.