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Pregnancy exacerbates complications of acquired hemophilia in a patient with systemic lupus erythematosus.

Sebastian A, Misterska-Skóra M, Podolak-Dawidziak M, Szmyrka-Kaczmarek M, Sebastian M, Wiland P - Postepy Dermatol Alergol (2015)

View Article: PubMed Central - PubMed

Affiliation: Department of Rheumatology and Internal Medicine, Wroclaw Medical University, Wroclaw, Poland. Head of the Department: Prof. Piotr Wiland MD, PhD.

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Acquired hemophilia (AH) is a rare disease characterized by the production of antibodies directed against blood coagulation fVIII leading to coagulation disorders due to lowering of its activity... Laboratory testing revealed significantly prolonged APTT (121 s, normal value 25–37 s), leukopenia (3.33 × 10/µl, normal value 4–10 × 10/µl), lymphopenia (0.93 × 10/µl, normal value 1.5–3.5 × 10/µl), anemia (hemoglobin level 10.9 g/dl, normal value 12–16 g/dl), PLT count of 205 × 10/µl (normal value 140–440 × 10/µl), decreased levels of both complement C3 (0.57, normal value 0.9–1.8) and C4 (< 0.08, normal value 0.1–0.4) components, the presence of antinuclear antibodies (ANA) (1: 320) which exhibited a granular pattern of fluorescence and SS-A and Ro52 antibodies... It is not recommended to assess this parameter during thrombotic episodes due to the presence of acute inflammatory reactions (including fVIII) what may influence the APTT results... Lupus anticoagulant testing was performed twice during and after withdrawal of LMWH treatment and neither the presence of anti-β2GPI nor LA were confirmed... The LMWH treatment caused the hemorrhagic diathesis which did not disappear after its withdrawal... There were reported cases of AH in the course of SLE in pregnancy and puerperium in both active and inactive SLE... The patient got pregnant despite the fact that effective contraception was recommended and retroperitoneal bleeding occurred... Pregnancy in patients with AH is particularly dangerous to both the mother and the fetus because the hemorrhage can occur not only in the mother but antibodies against coagulant factors can pass through the placental barrier and lead also to massive bleeding in the fetus... Treatment consists of a high dose GC as the first-line therapy e.g. prednisone at a dose of 1 mg/kg for several weeks with a further dose reduction... It was found that combined therapy with prednisone plus cyclophosphamide at an oral dose of 2 mg/kg/day may be effective in patients with no response to steroid monotherapy... Firstly, at the beginning of the disease the diagnosis of APS complicated with PE was very probable due to its symptoms (dyspnea, hemoptysis, chest pain) with prolonged APTT and characteristic features in spiral computed angiotomography of the chest... Antiphospholipid syndrome is a more common complication of SLE than AH because antiphospholipid antibodies are found in about 30% of patients with SLE... In conclusion, APTT should be monitored in patients with SLE associated with symptomatic hemorrhagic diathesis.

No MeSH data available.


Related in: MedlinePlus

Urticaria on the back
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Figure 0002: Urticaria on the back

Mentions: The patient was admitted to the Department of Rheumatology in September 2010 with large and multiple areas of ecchymoses in the upper and lower extremities (Figures 1 A, B). During hospitalization there appeared bilateral episcleritis which was treated topically and recurring skin rash in the form of urticaria (Figure 2). Laboratory testing revealed significantly prolonged APTT (121 s, normal value 25–37 s), leukopenia (3.33 × 103/µl, normal value 4–10 × 103/µl), lymphopenia (0.93 × 103/µl, normal value 1.5–3.5 × 103/µl), anemia (hemoglobin level 10.9 g/dl, normal value 12–16 g/dl), PLT count of 205 × 103/µl (normal value 140–440 × 103/µl), decreased levels of both complement C3 (0.57, normal value 0.9–1.8) and C4 (< 0.08, normal value 0.1–0.4) components, the presence of antinuclear antibodies (ANA) (1: 320) which exhibited a granular pattern of fluorescence and SS-A and Ro52 antibodies. The level of ds-DNA was 18.35 IU/ml (normal value < 100). The APS and PE were excluded on the basis of laboratory and immunological testing (lupus anticoagulant (LA), antibodies against β2-glycoprotein (anti-β2GPI), anticardiolipin antibodies (ACA)) and a control spiral computed angiotomography of the chest. Treatment with LMWH was stopped. A slight reduction in APTT to 115 s was achieved. Physical examination revealed a smaller number and size of areas of ecchymoses. The diagnosis of AH was established on the basis of low coagulation fVIII level (< 5%, normal value 50–150%). The level of coagulation fVIII inhibitor measured with Bethesda method was 614.4 IU/ml (the Bethesda units are used to evaluate the concentration of fVIII inhibitor what is based on establishing such dilution when the activity of fVIII is 50%. The higher concentration of the inhibitor, the higher the number of Bethesda units). The treatment consisted of oral methylprednisolone 20 mg/day, chloroquine 250 mg/day and cyclosporine 200 mg/day what led to clinical improvement. A prolonged APTT was still observed in laboratory tests.


Pregnancy exacerbates complications of acquired hemophilia in a patient with systemic lupus erythematosus.

Sebastian A, Misterska-Skóra M, Podolak-Dawidziak M, Szmyrka-Kaczmarek M, Sebastian M, Wiland P - Postepy Dermatol Alergol (2015)

Urticaria on the back
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4495105&req=5

Figure 0002: Urticaria on the back
Mentions: The patient was admitted to the Department of Rheumatology in September 2010 with large and multiple areas of ecchymoses in the upper and lower extremities (Figures 1 A, B). During hospitalization there appeared bilateral episcleritis which was treated topically and recurring skin rash in the form of urticaria (Figure 2). Laboratory testing revealed significantly prolonged APTT (121 s, normal value 25–37 s), leukopenia (3.33 × 103/µl, normal value 4–10 × 103/µl), lymphopenia (0.93 × 103/µl, normal value 1.5–3.5 × 103/µl), anemia (hemoglobin level 10.9 g/dl, normal value 12–16 g/dl), PLT count of 205 × 103/µl (normal value 140–440 × 103/µl), decreased levels of both complement C3 (0.57, normal value 0.9–1.8) and C4 (< 0.08, normal value 0.1–0.4) components, the presence of antinuclear antibodies (ANA) (1: 320) which exhibited a granular pattern of fluorescence and SS-A and Ro52 antibodies. The level of ds-DNA was 18.35 IU/ml (normal value < 100). The APS and PE were excluded on the basis of laboratory and immunological testing (lupus anticoagulant (LA), antibodies against β2-glycoprotein (anti-β2GPI), anticardiolipin antibodies (ACA)) and a control spiral computed angiotomography of the chest. Treatment with LMWH was stopped. A slight reduction in APTT to 115 s was achieved. Physical examination revealed a smaller number and size of areas of ecchymoses. The diagnosis of AH was established on the basis of low coagulation fVIII level (< 5%, normal value 50–150%). The level of coagulation fVIII inhibitor measured with Bethesda method was 614.4 IU/ml (the Bethesda units are used to evaluate the concentration of fVIII inhibitor what is based on establishing such dilution when the activity of fVIII is 50%. The higher concentration of the inhibitor, the higher the number of Bethesda units). The treatment consisted of oral methylprednisolone 20 mg/day, chloroquine 250 mg/day and cyclosporine 200 mg/day what led to clinical improvement. A prolonged APTT was still observed in laboratory tests.

View Article: PubMed Central - PubMed

Affiliation: Department of Rheumatology and Internal Medicine, Wroclaw Medical University, Wroclaw, Poland. Head of the Department: Prof. Piotr Wiland MD, PhD.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Acquired hemophilia (AH) is a rare disease characterized by the production of antibodies directed against blood coagulation fVIII leading to coagulation disorders due to lowering of its activity... Laboratory testing revealed significantly prolonged APTT (121 s, normal value 25–37 s), leukopenia (3.33 × 10/µl, normal value 4–10 × 10/µl), lymphopenia (0.93 × 10/µl, normal value 1.5–3.5 × 10/µl), anemia (hemoglobin level 10.9 g/dl, normal value 12–16 g/dl), PLT count of 205 × 10/µl (normal value 140–440 × 10/µl), decreased levels of both complement C3 (0.57, normal value 0.9–1.8) and C4 (< 0.08, normal value 0.1–0.4) components, the presence of antinuclear antibodies (ANA) (1: 320) which exhibited a granular pattern of fluorescence and SS-A and Ro52 antibodies... It is not recommended to assess this parameter during thrombotic episodes due to the presence of acute inflammatory reactions (including fVIII) what may influence the APTT results... Lupus anticoagulant testing was performed twice during and after withdrawal of LMWH treatment and neither the presence of anti-β2GPI nor LA were confirmed... The LMWH treatment caused the hemorrhagic diathesis which did not disappear after its withdrawal... There were reported cases of AH in the course of SLE in pregnancy and puerperium in both active and inactive SLE... The patient got pregnant despite the fact that effective contraception was recommended and retroperitoneal bleeding occurred... Pregnancy in patients with AH is particularly dangerous to both the mother and the fetus because the hemorrhage can occur not only in the mother but antibodies against coagulant factors can pass through the placental barrier and lead also to massive bleeding in the fetus... Treatment consists of a high dose GC as the first-line therapy e.g. prednisone at a dose of 1 mg/kg for several weeks with a further dose reduction... It was found that combined therapy with prednisone plus cyclophosphamide at an oral dose of 2 mg/kg/day may be effective in patients with no response to steroid monotherapy... Firstly, at the beginning of the disease the diagnosis of APS complicated with PE was very probable due to its symptoms (dyspnea, hemoptysis, chest pain) with prolonged APTT and characteristic features in spiral computed angiotomography of the chest... Antiphospholipid syndrome is a more common complication of SLE than AH because antiphospholipid antibodies are found in about 30% of patients with SLE... In conclusion, APTT should be monitored in patients with SLE associated with symptomatic hemorrhagic diathesis.

No MeSH data available.


Related in: MedlinePlus