Hemorrhagic Thrombocythemia; Control of Postsplenectomy Thrombocytosis with Melphalan

Bensinger, Thomas A.; Logue, Gerald L.; Rundles, R. Wayne

doi:10.1182/blood.v36.1.61.61

Cited by 39 publications

(5 citation statements)

References 15 publications

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“…Platelets in excess of 400 × 10 9 /L, and an increase of clustered enlarged megakaryocytes in a bone marrow biopsy material was found to be diagnostic for ET and excluded reactive thrombocytosis. On top of the clinical PVSG criteria for PV [46] we introduced in 1980 bone marrow histopathology and erythrocyte count above 6 × 10 12 /L proposed by Dameshek in 1940 [94] as specific clues to the diagnosis of PV to clearly differentiate PV from all variant of primary and secondary erythrocytosis. The 1980 RCP modifications of the 1975 PVSG criteria for PV include 4 main changes [13].…”

Section: Discussionmentioning

confidence: 99%

“…Coronary heart disease (not specified) 2 [31][32][33][34][35][36][37][38][39][40][41][42][43][44][45]) primary hemorrhagic thrombocythemia, [28,[46][47][48] primary thrombocythemia [49], essential thrombocythemia [50][51][52][53], persistent thrombocythemia [54], thrombocythemia [55][56][57], postsplenectomy thrombocythemia [58], thrombocytosis [59], hemorrhagic thrombocytosis [60,61], myelose hyperthrombocytaire [62] and hyperplaquettose [63].…”

Section: A Vascular Manifestations As the Intial Symptom Of Pv In 26mentioning

confidence: 99%

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Erythromelalgic Thrombotic Thrombocythemia (ETT) and Hemorrhagic Trombocythemia (HT) in Patients with Essential Thrombocythaemia (ET) and Polycythaemia Vera (PV)

Michiels¹

2014

J Hematol Thrombo Dis

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Citation: Michiels JJ (2014) Erythromelalgic Thrombotic Thrombocythemia (ETT) and Hemorrhagic Trombocythemia (HT) in Patients with Essential Thrombocythaemia (ET) and Polycythaemia Vera (PV). J Hematol Thromb Dis 2: 169. AbstractWe analysed between 1908 and 1985 of two hundred cases of thrombocythemia from the literature between 1908 and 1985. Erythromelalgic, acrocyanotic ischemia and digital gangrene, cerebral or coronary ischemic events (erythromelalgic thrombotic thrombocyhemia: ETT) in 99 thrombocythemia patients (essential thrombocythemia: ET N=67 and polycythemia vera: PV N=32) occured at platelet counts in excess of 400 × 10 9 /L. Hemorrhagic complications at time of presentation of hemorrhagic thrombocythemia (HT) in 100 HT patients included melana and/or hematemesis, skin bleedings (bruises, subcutaneous hematomas, painfull hematomas after trauma, ecchymoses, but not petechiae, epistaxis and gum bleeding and secondary bleeding after trauma or surgery. ETT occurred at an early stage of thrombocythemia with a mean platelet count of 1110+477 × 10 9 /L, whereas HT occurred at significantly higher platelet counts in excess of 1000 × 10 9 /L with a mean platelet count of 2016+1070 × 10 9 /L. HT patients frequently had a previous history of ETT or paradoxical occurrences of both thrombosis and bleeding (ETT/PHT). The degree of thrombocythosis determined the sequential occurrence of ETT, HT in ET and PV patients. Hypersensitive thrombocythemic platelets (sticky platelets) are involved in the etiology of platelet-mediated thrombosis at platelet counts above 400 × 10 9 /L whereas bleedings at high platelet count is related to an acquired von Willebrand syndrome processes on top of a blood clot retraction disturbance with erythrocyte fall out, that causes painful subcutaneous hematomas with a central swelling (clot) after a blow, trauma and secondary bleeding after surgery in thrombocythemia patients. Jo u r n a l o f Hema to lo g y & Thro m b o e m bolic D is e a s es

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Section: Discussionmentioning

confidence: 99%

Section: A Vascular Manifestations As the Intial Symptom Of Pv In 26mentioning

confidence: 99%

Erythromelalgic Thrombotic Thrombocythemia (ETT) and Hemorrhagic Trombocythemia (HT) in Patients with Essential Thrombocythaemia (ET) and Polycythaemia Vera (PV)

Michiels¹

2014

J Hematol Thrombo Dis

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“…On rare occasions, however, the platelet count may remain persistently elevated and give rise to hemorrhagic and/or thrombotic complications. This type of thrombocythemia has not been differentiated from primary thrombocythemia (6)(7)(8)(9)(10)(11)(12); in fact, some authors postu'ate that splenectomy unmasks or aggravates an underlying disease process (7,10,(13)(14)(15)(16)(17). Other authors postulate that the thrombocythemia is a direct consequence of splenectomy (2,4,11,(18)(19)(20)(21).…”

mentioning

confidence: 99%

Postsplenectomy Thrombocythemia: An Unmasked Myeloproliferative Disorder

Jamshidi¹,

Ansari²,

Garcia³

et al. 1973

J American Geriatrics Society

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Persistent postsplenectomy thrombocythemia should be differentiated from transient postsplenectomy thrombocytosis, as the former leads to hemorrhagic and thrombotic complications. The authors reviewed 29 cases of "postsplenectomy thrombocythemia" collected from the literature and added 2 of their own. The data on these 31 cases indicate that the disease should be diagnosed and treated as primary thrombocythemia, present in masked form before splenectomy.

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“…Melphalan has been used in hemorrhagic thrombocythemia post-splenectomy as a cytoreductive agent and was proven to be effective in controlling the platelet proliferation thereby reducing the bleeding complications in some situations [ 57 ]. The role of Melphalan has not been investigated in depth over the ensuing decades and could be studied further in patients with RT to prevent thrombotic events and/or be used as a more effective agent compared to hydroxyurea or anagrelide.…”

Section: Discussionmentioning

confidence: 99%

Fatal pulmonary embolism following splenectomy in a patient with Evan’s syndrome: case report and review of the literature

2017

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BackgroundEvans syndrome (ES) is a rare disease characterized by simultaneous or sequential development of autoimmune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP) with or without immune neutropenia. Splenectomy is one of the treatment options for disease refractory to medical therapy. Venous thromboembolism (VTE) following splenectomy for hematological diseases has an incidence of 10%.Case presentationHere we describe a case report of a young patient hospitalized with severe hemolytic anemia with Hgb 4.8 g/dl. He developed thrombocytopenia with platelet nadir of 52,000/mm3, thus formally diagnosed with ES. He failed standard medical therapy. He underwent splenectomy and had a fatal outcome. Autopsy confirmed the cause of death as pulmonary embolism (PE).ConclusionsThis case report and review of the literature highlight important aspects of the association between VTE, splenectomy, and hemolytic syndromes including the presence of thrombocytopenia. The burden of the disease is reviewed as well as various pathophysiologic mechanisms contributing to thromboembolic events in these patients and current perioperative prophylactic anticoagulation strategies. Despite an advancing body of literature increasing awareness of VTE following splenectomy, morbidity and mortality remains high. Identifying high risk individuals for thromboembolic complications from splenectomy remains a challenge. There are no consensus guidelines for proper perioperative and post-operative anti-coagulation. We encourage future research to determine which factors might be playing a role in increasing the risk for VTE in real time with hope of forming a consensus to guide management.

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Hemorrhagic Thrombocythemia; Control of Postsplenectomy Thrombocytosis with Melphalan

Cited by 39 publications

References 15 publications

Erythromelalgic Thrombotic Thrombocythemia (ETT) and Hemorrhagic Trombocythemia (HT) in Patients with Essential Thrombocythaemia (ET) and Polycythaemia Vera (PV)

Erythromelalgic Thrombotic Thrombocythemia (ETT) and Hemorrhagic Trombocythemia (HT) in Patients with Essential Thrombocythaemia (ET) and Polycythaemia Vera (PV)

Postsplenectomy Thrombocythemia: An Unmasked Myeloproliferative Disorder

Fatal pulmonary embolism following splenectomy in a patient with Evan’s syndrome: case report and review of the literature

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