Amegakaryocytic Thrombocytopenia in Systemic Lupus Erythematosus

Griner, Paul F.

doi:10.1001/archinte.1970.00310020134020

Cited by 33 publications

(4 citation statements)

References 20 publications

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“…Amegakaryocytic thrombocytopenia may be a primary disorder itself 10 or may be seen in aplastic anemia, 11 preleukemia, 11 and in systemic lupus erythematosus. 12-14 It has also been reported in patients with Graves’ disease treated with radioiodine in the past, 15 in congenital rubella, dengue fever, nutritional B 12 deficiency, ethanol abuse, and certain congenital disorders like the thrombocytopenia-absent radius (TAR) syndrome.…”

Section: Discussionmentioning

confidence: 99%

Myelodysplastic Syndrome Presenting as Amegakaryocytic Thrombocytopenia in a Collodion Baby

Pakra

Jabri

Hanafy

2015

Journal of Investigative Medicine High Impact Case Reports

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We report a rare case of myelodysplastic syndrome that presented early as amegakaryocytic thrombocytopenia in a collodion baby, which is a rare congenital disorder characterized by thick, taut membrane resembling oiled parchment or collodion, which is subsequently shed. To our knowledge, this is the first reported case of a collodion baby who presented with amegakaryocytic thrombocytopenia and who has a significant family history of the same condition. We document the rarity of this possible association and also the need for further study to establish whether a causal relationship exists.

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Section: Discussionmentioning

confidence: 99%

Myelodysplastic Syndrome Presenting as Amegakaryocytic Thrombocytopenia in a Collodion Baby

Pakra

Jabri

Hanafy

2015

Journal of Investigative Medicine High Impact Case Reports

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“…Most cases of acquired amegakaryocytic thrombocytopenia in humans are immune-mediated, such as systemic lupus erythematosus (Griner and Hoyer 1970, Worman and others 1982, Skurai and others 1984, Hoffman and others 1985, Slater and others 1985, Gewirtz and others 1986, Nagasawa and others 1986, Benedetti and others 1994, Lu and others 1999, Zafar and others 1999. None of the four dogs reported here had positive ANA titres or clinical signs compatible with systemic lupus erythematosus, although dogs 1 and 2 did have evidence of idiopathic immune-mediated disease.…”

Section: Discussionmentioning

confidence: 99%

Acquired amegakaryocytic thrombocytopenia — four cases and a literature review

Lachowicz¹,

Post²,

Moroff

et al. 2004

J of Small Animal Practice

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Acquired amegakaryocytic thrombocytopenla was diagnosed in four dogs. Initial platelet counts in all four dogs were less than 50,000 x 10(9)/litre and initial bone marrow examinations revealed megakaryocytic hypoplasia with minimal changes in the erythroid and myeloid cell lines. Two dogs had evidence of idiopathic immune-mediated disease and two dogs had evidence of associated infectious disease. One dog had a positive antibody titre to Borrella burgdorferi, and one dog had positive titres to both Ehrlichia canis and B. burgdorferi. Treatment consisted of prednisone and cyclophosphamide for the dogs with presumptive immune-mediated disease, and prednisone and tetracycline for the dogs with positive antibody titres to the Infectious organisms. Both dogs with evidence of associated infectious disease responded to treatment. A postmortem examination did not reveal the underlying aetiology in the two dogs with presumptive idiopathic immune-mediated disease.

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“…It may be an early manifestation of an intrinsic stem cell abnormality, as some patients subsequently develop aplastic anemia, myelodysplastic syndrome (MDS) and acute leukemia [1,4]. However, it has also been reported with systemic lupus erythematosus (SLE) [5][6][7] and vitamin B 12 deficiency [8], suggesting an underlying immune aberration in some cases. Although there were occasional spontaneous remissions, most sustained ones were in patients receiving immunosuppressive therapy, such as corticosteroids [1,7,9], cyclosporin A [4,12], antithymocyte globulin (ATG) [9][10][11], danazol [13], immunoglobulin (IVIG) [14], or cyclophosphamide [15].…”

Section: Introductionmentioning

confidence: 99%

Successful treatment of acquired amegakaryocytic thrombocytopenic purpura refractory to corticosteroids and intravenous immunoglobulin with antithymocyte globulin and cyclosporin

2008

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Four patients with acquired amegakaryocytic thrombocytopenic purpura, who had failed corticosteroids, intravenous immunoglobulin and cyclophosphamide therapy, were treated with antithymocyte globulin, followed by cyclosporin. Three patients achieved complete remission in 28-178 days and the response duration was 16-60 months from the beginning of treatment. One patient achieved a partial response for 2 months followed by myelodysplastic syndrome 5 months later. He died in 9 months due to intracerebral bleeding. Marrow cytogenetics showed 47, XY, +21.

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Amegakaryocytic Thrombocytopenia in Systemic Lupus Erythematosus

Cited by 33 publications

References 20 publications

Myelodysplastic Syndrome Presenting as Amegakaryocytic Thrombocytopenia in a Collodion Baby

Myelodysplastic Syndrome Presenting as Amegakaryocytic Thrombocytopenia in a Collodion Baby

Acquired amegakaryocytic thrombocytopenia — four cases and a literature review

Successful treatment of acquired amegakaryocytic thrombocytopenic purpura refractory to corticosteroids and intravenous immunoglobulin with antithymocyte globulin and cyclosporin

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