Late Appearance of Thrombotic Thrombocytopenic Purpura after Autoimmune Hemolytic Anemia and in the Course of Chronic Autoimmune Thrombocytopenic Purpura: Two Case Reports

Krupsky, Meir; Sarel, Raanana; Hurwitz, Nina; Resnitzky, P

doi:10.1159/000204876

Cited by 14 publications

(12 citation statements)

References 19 publications

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“…Similarly, TTP has been associated with au-toimmune disorders other than ITP [18]. For example, Krupsky et al [11] described a patient with autoimmune hemolytic anemia who developed TTP.…”

Section: Discussionmentioning

confidence: 99%

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Four patients with both thrombotic thrombocytopenic purpura and autoimmune thrombocytopenic purpura: The concept of a mixed immune thrombocytopenia syndrome and indications for plasma exchange

Baron

Martin

Sucharetza

et al. 2001

J of Clinical Apheresis

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Autoimmune thrombocytopenic purpura (ATP) and thrombotic thrombocytopenic purpura (TTP) are each well recognized clinical syndromes which may appear as single episodes or may have chronic relapsing courses. We present four patients negative for human immunodeficiency virus (HIV) infection who appear to have both diagnoses with either concomitant or intermingled episodes, and we review seven additional patients reported in the literature with similar features. All four of our patients are female, two have underlying connective tissue disorders, and their ATP processes came to our attention because of incomplete response of the platelet count to plasma exchange therapy (PEX) during a TTP phase (Cases 1 and 2) or development of thrombocytopenia in the absence of microangiopathy on the background of prior typical TTP episodes (Cases 3 and 4). Recognition of the ATP diagnosis in each case resulted in discontinuation of PEX (Cases 1 and 2) or not instituting PEX (Cases 3 and 4). In each instance, a satisfactory rise in platelet count followed treatment for ATP. Based upon this experience, we conclude that some individuals may have a mixed immune thrombocytopenia syndrome; careful analysis of the mechanism of thrombocytopenia, especially in recurrent episodes and in patients who respond incompletely to PEX for TTP, is important when deciding whether to initiate or continue PEX, or to consider therapies appropriate for other mechanisms of thrombocytopenia.

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“…Similarly, TTP has been associated with au-toimmune disorders other than ITP [18]. For example, Krupsky et al [11] described a patient with autoimmune hemolytic anemia who developed TTP.…”

Section: Discussionmentioning

confidence: 99%

“…Assays for antibodies to metalloproteinase, if and when readily available, and antiplatelet antibodies may provide con®rmatory retrospective evidence. Splenectomy may be of long-term bene®t for either condition, although it is of interest that three patients [9,11,13] developed TTP following splenectomy for ITP (Table 1).…”

Section: Discussionmentioning

confidence: 99%

Four patients with both thrombotic thrombocytopenic purpura and autoimmune thrombocytopenic purpura: The concept of a mixed immune thrombocytopenia syndrome and indications for plasma exchange

Baron

Martin

Sucharetza

et al. 2001

J of Clinical Apheresis

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“…His patient had a positive Coombs’ test and some features of TTP (including fever, recurrent neurologic symptoms, and numerous erythrocyte fragments in the peripheral blood smear), which was later confirmed by necropsy findings. In 1991, Krupsky et al [19]reported an association between TTP and two autoimmune hematological conditions, AIHA and ITP. In their report of TTP associated with AIHA, those disorders occurred 5 years apart in a 35-year-old male, with AIHA occurring first.…”

Section: Discussionmentioning

confidence: 99%

Warm-Antibody Autoimmune Hemolytic Anemia Developing after Thrombotic Thrombocytopenic Purpura

et al. 2002

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Thrombotic thrombocytopenic purpura (TTP) and warm-antibody autoimmune hemolytic anemia (AIHA) are uncommon diseases. Although TTP has been increasingly described in association with autoimmune antibodies, there are very few reports of the association with autoimmune hematological conditions, including idiopathic thrombocytopenic purpura and AIHA. Here we describe a patient with classic manifestations of TTP, who was successfully treated with plasma exchange. A few weeks later, she developed warm-antibody AIHA, which responded promptly to prednisone.

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“…It has been well documented that some patients with ITP may develop TTP, or TTP may relapse as ITP [13][14][15]. These overlapping syndromes are not yet well recognized but are more frequently described in patients with HIV and HTLV-I infection [16].…”

Section: Itp/ttp Overlapping Syndromes: Platelet Consumption In the Mmentioning

confidence: 99%

Idiopathic thrombocytopenic purpura: Pathophysiology and management

Ahn

Horstman

2002

Int J Hematol

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Our understanding of the pathophysiology of ITP owes to pioneering work of W J Harrington in 1951, delineating the immunologic nature of platelet destruction. In ITP, antibody-coated platelets are destroyed by macrophages of RES. However, other mechanisms are also implicated: C-mediated platelet lysis and newly described C-independent peroxide injury. Both induce platelet fragmentation and lysis, generating procoagulant platelet microparticles (PMP). A third mechanism of platelet consumption in the microvasculature is proposed, based on overlapping syndromes of ITP and TTP in some patients. In assessing hemostasis in ITP, platelet counts alone is not sufficient. Evaluation of platelet clumping, giant platelets, and platelet activation, marked by increased PMP is useful. Patients with platelet activation or giant platelets bleed less and detection of clumping prevents unwarranted therapy. Thrombotic complications may develop in ITP. A syndrome, characterized by recurrent TIA-like symptoms, progressive memory loss due to ischemic small vessel disease is described. The management of ITP should include the search for and elimination of underlying causes and careful evaluation of hemostasis. Therapy is divided into definitive vs symptomatic measures. The former including splenectomy, danazol, chemotherapy offers lasting remission after therapy was stopped, while the later including glucocorticoids, gammaglobuin, antiD antibodies and others increases platelet counts but seldom sustains remission upon withdrawal. Danazol therapy is up-dated since it is an effective and safe definite measure in ITP.

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Late Appearance of Thrombotic Thrombocytopenic Purpura after Autoimmune Hemolytic Anemia and in the Course of Chronic Autoimmune Thrombocytopenic Purpura: Two Case Reports

Cited by 14 publications

References 19 publications

Four patients with both thrombotic thrombocytopenic purpura and autoimmune thrombocytopenic purpura: The concept of a mixed immune thrombocytopenia syndrome and indications for plasma exchange

Four patients with both thrombotic thrombocytopenic purpura and autoimmune thrombocytopenic purpura: The concept of a mixed immune thrombocytopenia syndrome and indications for plasma exchange

Warm-Antibody Autoimmune Hemolytic Anemia Developing after Thrombotic Thrombocytopenic Purpura

Idiopathic thrombocytopenic purpura: Pathophysiology and management

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