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• W1973733024 abstract "Dear Sir, A 21-year-old previously healthy Guyanese student with no significant past medical history was referred to our emergency department in July 2001 complaining of generalized headaches for 4 months and a 24-h history of severe headache, nausea, vomiting, L facial droop and left arm weakness. On history taking, he recounted that 10 months previously, he began experiencing neck pains and increasingly frequent headaches. He sought help from a chiropractor in March 2001 and following his first neck manipulation developed a generalized seizure. He was immediately admitted to a community hospital where a brain computed tomography (CT) scan demonstrated a small right frontal intracerebral hemorrhage. He had been transferred to Neurosurgery at our hospital and a magnetic resonance imaging (MRI) of the brain showed slow flow through the right transverse sinus. In June 2001, a transfemoral venogram demonstrated complete occlusion of the right transverse sinus. No specific therapy was prescribed at this time with the exception of Dilantin. He had been discharged with outpatient follow-up with the thrombophilia and neurosurgical services. On examination during this particular presentation (July 2001) he had new left hemiparesis, left facial nerve palsy and splenomegaly. MRI and cerebral angiogram confirmed superior sagittal and right transverse sinus thromboses and small subacute and chronic intracerebral bleeds consistent with cerebral vein infarcts. CT abdomen demonstrated only isolated homogeneous mild/moderate splenomegaly (15.4 cm). Blood work, including complete blood count (CBC), lytes, blood urea nitrogen (BUN), creatinine, liver and calcium profile were all within normal limits. On four occasions, his mean corpuscular volume (MCV) was noted to be borderline low at 76–77 (normal 78–96 FL), but a ferritin and Hg electrophoresis were normal and alpha thallesemia trait was entertained, although genotyping was not performed. An extensive thrombophilia screen was normal (ATIII, proteins C, S, factor V Leiden, prothrombin variant, homocysteine and lupus anticoagulant) with the exception of a slightly increased IgM anticardiolipin antibody (ACA) of 17 MPL (normal 0–11). There was no family history of thrombosis. He was anticoagulated with heparin followed by warfarin. The patient slowly recovered and was discharged home 3 weeks later with follow-up. The patient remained well for 3 months but re-presented to the emergency department complaining of a painful right foot and bluish discoloration of his right 3–5 toes. Doppler and digital plethysmography showed no evidence of large vessel occlusion and he was treated for vasospasm with prostaglandin infusion, calcium channel blockers and nitrates with modest effect. A vasculitis work-up was found to be negative and no vegetations were detected on 2-dimensional echo of his heart. During this admission, his ACA IgG had risen to 43 GPL units (0–20) and his IgM ACA remained elevated at 18 MPL units (0–11). Antiphospholipid antibody syndrome (APLA) was considered, ASA was added and the patient's anticoagulation was increased (target INR 2.5–3.5). On this occasion, his MCV was consistently low at 72–76, and cells were noted to be hypochromic and microcytic with occasional tear drops on blood film, but ferritin remained in the normal range, albeit lower than previously. Hg and WBC were consistently normal, although his RBC count was noted to be elevated on several occasions ranging from 6.35 to 6.7 × 1012 L−1 (normal 4–6 × 1012 L−1). A repeat ultrasound was performed upon discharge and hepatomegaly (15.2 cm) and persistent splenomegaly (19.3 cm) were documented. Peripheral blood was sent for CFU-GEMM colony growth by plating mononuclear cells with and without erythropoietin at 2 U mL−1 (methods previously published) [1Messner H.A. Jamal N. Cell culture assessment of hematopoietic progenitors in bone marrow transplantation.J Hematother. 1993; 2: 19-25Crossref Scopus (3) Google Scholar, 2Cowan D.H. Messner H.A. Jamal N. Aye M.T. Smiley R.K. Spontaneous remission of polycythemia vera: clinical and cell culture characteristics.Am J Hematol. 1994; 46: 54-6Crossref Scopus (5) Google Scholar]. These results are presented in Table 1 and supported spontaneous erythroid colony growth. A bone marrow aspirate and biopsy were then done and demonstrated again spontaneous erythroid colonies and findings consistent with a myeloproliferative disorder (MPD) such as polycythemia vera (erythroid and megakaryocyte hyperplasia, increased reticulin, absent iron stores) evolving into the spent phase (Fig. 1).Table 1Serial spontaneous erythroid colony results on peripheral blood and marrowDateSample typeCells plated (cm−3)BFU-E without erythropoietinBFU-E with erythropoietin13 March 2002Peripheral blood1 × 105451259 July 2002Bone marrow5 × 10432969 July 2002Peripheral blood1 × 10580166BFU-E, Blast forming units erythroid. Open table in a new tab BFU-E, Blast forming units erythroid. The patient is currently asymptomatic and thrombosis-free for 10 months on warfarin and ASA. His blood counts remain in the normal range. ACA IgG and IgM levels have also normalized, 8 months following the ischemic foot episode. He has just started alpha-interferon and will be evaluated for an allogeneic marrow transplant. Both antiphospholipid syndromes and MPD are included in the small list of prothrombotic states in which thrombosis occurs within both the venous and arterial beds [3Levine J.S. Branch D.W. Rauch J. The antiphospholipid syndrome.N Engl J Med. 2002; 346: 752-63Crossref PubMed Scopus (1315) Google Scholar]. Polycythemia rubra vera (PV) is a clonal MPD classically associated with increased red cell mass, leukocytosis, thrombocytosis, splenomegaly, thromboses and bleeding. Idiopathic arterial and venous thromboses have been reported to be the presenting feature of previously undiagnosed MPD with common sites including the portal, hepatic or splanchnic veins [4Valla D. Casadevall N. Lacombe C. Varet B. Goldwasser E. Franco D. Maillard J.N. Pariente E.A. Leporrier M. Rueff B. et al.Primary myeloproliferative disorder and hepatic vein thrombosis. A prospective study of erythroid colony formation in vitro in 20 patients with Budd–Chiari syndrome.Ann Intern Med. 1985; 103: 329-34Crossref PubMed Google Scholar, 5De Stefano V. Teofili L. Leone G. Michiels J.J. Spontaneous erythroid colony formation as the clue to an underlying myeloproliferative disorder in patients with Budd–Chiari syndrome or portal vein thrombosis.Semin Thromb Hemost. 1997; 23: 411-8Crossref PubMed Google Scholar, 6Pagliuca A. Mufti G.J. Janossa-Tahernia M. Eridani S. Westwood N.B. Thumpston J. Sawyer B. Sturgess R. Williams R. In vitro colony culture and chromosomal studies in hepatic and portal vein thrombosis—possible evidence of an occult myeloproliferative state.Q J Med. 1990; 76: 981-9PubMed Google Scholar]. Recent stroke registries (HART) have shown that 0–7% of cerebral infarcts may be attributed to hematological causes. Indeed, two patients described by Haan et al. [7Haan J. Caekebeke J.F. Van Der Meer F.J. Wintzen A.R. Cerebral venous thrombosis as presenting sign of myeloproliferative disorders.J Neurol Neurosurg Psychiatry. 1988; 51: 1219-20Crossref PubMed Google Scholar] presented with cerebral vein thrombosis and were diagnosed with MPD based on histological findings. Spontaneous erythroid colonies from peripheral blood or marrow samples in erythropoietin-poor media are considered a usual feature of PV, and can be detected before any overt manifestations of the disease occur [4Valla D. Casadevall N. Lacombe C. Varet B. Goldwasser E. Franco D. Maillard J.N. Pariente E.A. Leporrier M. Rueff B. et al.Primary myeloproliferative disorder and hepatic vein thrombosis. A prospective study of erythroid colony formation in vitro in 20 patients with Budd–Chiari syndrome.Ann Intern Med. 1985; 103: 329-34Crossref PubMed Google Scholar, 5De Stefano V. Teofili L. Leone G. Michiels J.J. Spontaneous erythroid colony formation as the clue to an underlying myeloproliferative disorder in patients with Budd–Chiari syndrome or portal vein thrombosis.Semin Thromb Hemost. 1997; 23: 411-8Crossref PubMed Google Scholar, 6Pagliuca A. Mufti G.J. Janossa-Tahernia M. Eridani S. Westwood N.B. Thumpston J. Sawyer B. Sturgess R. Williams R. In vitro colony culture and chromosomal studies in hepatic and portal vein thrombosis—possible evidence of an occult myeloproliferative state.Q J Med. 1990; 76: 981-9PubMed Google Scholar, 8Teofili L. De Stefano V. Leone G. Micalizzi P. Iovino M.S. Alfano G. Bizzi B. Hematological causes of venous thrombosis in young people: high incidence of myeloproliferative disorder as underlying disease in patients with splanchnic venous thrombosis.Thromb Haemost. 1992; 67: 297-301Crossref PubMed Scopus (52) Google Scholar]. This method has been reported to be particularly helpful in diagnosing cases of PV involving iron deficiency, where the usually elevated blood counts are not found [9Masters G.S. Baines P. Jacobs A. Erythroid colony growth from peripheral blood and bone marrow in polycythaemia.J Clin Pathol. 1990; 43: 937-41Crossref Scopus (21) Google Scholar], as in this case. The delay in diagnosing this patient's MPD is not surprising given his young age, consistently normal blood counts, and normal ferritins. Furthermore, we suspected alternative causes for his microcytosis prior to the bone marrow (alpha thal trait) and thrombosis (APLA syndrome). APLA syndrome is associated with arterial and venous thrombosis, including deep vein thrombosis and pulmonary embolism, premature coronary artery disease, premature cerebrovascular disease (including stroke, transient ischemic attack, cerebrovascular thrombotic stroke), and retinal arterial and venous occlusive disease. Thrombotic episodes associated with APLA syndrome may occur in vascular beds that are infrequently affected by other prothrombotic states with cutaneous manifestations that include distal cutaneous ischemia, infarcts of the skin, and acrocyanosis [3Levine J.S. Branch D.W. Rauch J. The antiphospholipid syndrome.N Engl J Med. 2002; 346: 752-63Crossref PubMed Scopus (1315) Google Scholar]. IgG, IgM and IgA anticardiolipin antibodies are all associated with thrombosis. Of patients with thrombosis and anticardiolipin antibodies 17% have isolated IgM idiotype [10Reyes H. Dearing L. Bick R.L. Shoenfeld Y. Peter J.B. Laboratory diagnosis of antiphospholipid syndromes.Clin Lab Med. 1995; 15: 85-108Abstract Full Text PDF PubMed Google Scholar]. There is no apparent association between the type of thrombotic event and type or titer of anticardiolipin antibody present [11Bick R.L. Baker W.F. Anticardiolipin antibodies and thrombosis.Hematol Oncol Clin North Am. 1992; 6: 1287-99Abstract Full Text PDF PubMed Google Scholar, 12Bick R.L. Antiphospholipid thrombosis syndromes.Clin Appl Thromb Hemost. 2001; 7: 241-58Crossref PubMed Google Scholar]. The relationship of his anticardiolipin antibodies to his cerebral venous thromboses and lower leg arterial insufficiency is not entirely clear. Although one study found elevated levels of ACAs in 45.6% of young stroke patients, correlation is not necessarily causality. Furthermore, low positive values may be transient or reactive phenomena [13Khamashta M.A. Hughes G.R. ACP Broadsheet, 136. February 1993. Detection and importance of anticardiolipin antibodies.J Clin Pathol. 1993; 46: 104-7Crossref PubMed Google Scholar]. The literature reports the temporal sequence of antibody levels following a thrombotic event as showing peaks in IgM at 5–7 days and in IgG in up to 2 weeks. The IgG peak occurred during his foot ischemia episode, but not following the cerebral vein thrombosis. Finally, the mere presence of antiphospholipid antibodies may be insufficient to generate thrombosis. A second ‘hit’ in combination may be required for thrombosis to occur. In this instance, the second hit may have been his MPD. Nevertheless, given the strong association of MPD with thrombosis, one cannot rule out the possibility that these anticardiolipin antibodies were unrelated, merely reactive phenomena. We believe this case is interesting to physicians for the following reasons: it reinforces the need to consider occult MPD in cases of unexplained venous and arterial thromboses, even in very young patients; it demonstrates that anticardiolipin antibodies and lupus anticoagulants may be reactive and transient phenomena and not necessarily the cause of thromboses, even when elevated; and finally, it illustrates an interesting and unusual case of a very young man with moderately advanced MPD being unmasked by idiopathic cerebral vein thromboses. The assistance of Hans Messner for providing the laboratory support and technology of the colony assays in gratefully acknowledged." @default.
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• W1973733024 date "2003-08-01" @default.
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• W1973733024 title "Cerebral vein thrombosis and right foot ischemia in a 21-year-old man" @default.
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