Thrombotic thrombocytopenic purpura complicating immune thrombocytopenic purpura—A case report

Changela, Avani; Jagarlamudi, Kuppuswamy; Villani, Gina; Alhuwalia, Meena; Elnawawi, Ashraf

doi:10.1002/ajh.21971

Cited by 5 publications

(2 citation statements)

References 1 publication

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“…The sequential occurrence of aTTP after ITP or vice versa is well recognized in the literature [ 12 , 13 , 14 ]. It has been described in HIV infected patients [ 15 , 16 , 17 ] and in patients with primary Sjögren’s syndrome or post-partum [ 18 , 19 ].…”

Section: Discussionmentioning

confidence: 99%

Concomitant presentation of thrombotic thrombocytopenic purpura, immune thrombocytopenia, and autoimmune hemolytic anemia in a patient with newly diagnosed systemic lupus erythematosus

Bruns,

Völker,

Klamroth

et al. 2023

CNCS

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Thrombocytopenia is always of concern when encountered in emergency settings. We report a case of a 29-year-old women in whom a unique constellation of hematological disorders occurred. The patient had been diagnosed with idiopathic immune thrombocytopenia (ITP) in 2007, with a history of several thrombocytopenic flares. She now presented with homonymous hemianopia accompanied by thrombocytopenia and microangiopathic hemolytic anemia (MAHA) and was soon after diagnosed with a posterior stroke. Symptoms were more reminiscent of acquired thrombotic thrombocytopenic purpura (aTTP) rather than ITP. Immediate treatment with plasma exchange and caplacizumab curtailed MAHA, and progressive ischemic disease was averted. ADAMTS-13 testing confirmed the diagnosis of immune-mediated aTTP. Repeated testing for ITP, however, also showed IgG-loaded thrombocytes with the former known anti-GPIIb/IIIa specificity. Furthermore, autoimmune hemolytic anemia (AIHA) could be detected by direct antiglobulin test showing IgG and complement loading of the patient’s erythrocytes. The autoimmune background of all three entities suggested an underlying systemic disease. Indeed, systemic lupus erythematosus (SLE) serology was strongly positive allowing for the diagnosis of SLE. ITP and AIHA as well as aTTP can be secondary to SLE, but emergence of all three disorders has not been reported at the same time.

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Section: Discussionmentioning

confidence: 99%

Concomitant presentation of thrombotic thrombocytopenic purpura, immune thrombocytopenia, and autoimmune hemolytic anemia in a patient with newly diagnosed systemic lupus erythematosus

Bruns,

Völker,

Klamroth

et al. 2023

CNCS

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show abstract

“…Sixteen publications [7][8][9][11][12][13][14][15][16][17][18][19][20][21][22][23] were evaluated in detail, and a total of 24 patients (including our patient) were included in this qualitative analysis. The patients' ages ranged from 9 to 72 years old, and the mean age was 36 years.…”

Section: Systemic Literature Reviewmentioning

confidence: 99%

Concurrence of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura: a case report and review of the literature

Lin

Huang

et al. 2023

J Med Case Reports

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Background Immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura are both causes of thrombocytopenia. Recognizing thrombotic thrombocytopenic purpura is crucial for subsequent treatment and prognosis. In clinical practice, corticosteroids and rituximab can be used to treat both immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura; plasma exchange therapy is the first-line treatment in thrombotic thrombocytopenic purpura, while corticosteroids are strongly recommended as first-line treatment in immune thrombocytopenic purpura. The differential diagnosis of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura is essential in clinical practice. However, case reports have suggested that immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura can occur concurrently. Case presentation We report the case of a 32-year-old Asian female without previous disease who presented with pancytopenia, concurrent with immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura. The morphology of the megakaryocytes in the bone marrow indicated immune-mediated thrombocytopenia. The patient received glucocorticoid treatment, and her platelet count increased; however, schistocytes remained high during the course of the therapy. Further investigations revealed ADAMTS13 activity deficiency and positive ADAMTS13 antibodies. The high titer of antinuclear antibody and positive anti-U1-ribonucleoprotein/Smith antibody indicated a potential autoimmune disease. However, the patient did not fulfill the current criteria for systemic lupus erythematosus or mixed connective tissue disease. The patient responded well to plasma exchange therapy, and her platelet count remained normal on further follow-up. Conclusions Concurrence of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura is rare, but clinicians should be aware of this entity to ensure prompt medical intervention. Most of the reported cases involve young women. Human immunodeficiency virus infection, pregnancy, and autoimmune disease are the most common underlying conditions.

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A case of systemic lupus erythematosus having concurrent Evans syndrome and acquired thrombotic thrombocytopenic purpura

Motoyama

Higuchi

Hirahara

et al. 2023

Modern Rheumatology Case Reports

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An 18-year-old Japanese woman with systemic lupus erythematosus (SLE) experienced dyspnoea, headache, tinnitus, and purpura for two weeks and was admitted to our hospital. The patient had been diagnosed with SLE and secondary immune thrombocytopenia eight years before and treated with high-dose prednisolone (PSL) and mycophenolate mofetil. Since the blood test on admission showed haemolytic anaemia with a positive direct Coombs test and anti-glycoprotein IIb/IIIa antibodies, the patient was initially diagnosed with Evans syndrome (ES). The patient was treated with pulse intravenous methylprednisolone followed by 45 mg/day prednisolone; however, the patient’s platelet count did not normalise. Based on a low level of a disintegrin-like metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS-13) activity and a high level of ADAMTS-13 inhibitors, a diagnosis of acquired thrombotic thrombocytopenic purpura (TTP) was confirmed. After undergoing therapeutic plasma exchange for six consecutive days, the patient’s platelet count recovered rapidly. Although concurrent acquired TTP and ES have not been reported previously, the findings from this case highlight the importance of measuring ADAMTS-13 activity and inhibitors to rule out acquired TTP, especially when ES is refractory to glucocorticoids.

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Thrombotic thrombocytopenic purpura complicating immune thrombocytopenic purpura—A case report

Cited by 5 publications

References 1 publication

Concomitant presentation of thrombotic thrombocytopenic purpura, immune thrombocytopenia, and autoimmune hemolytic anemia in a patient with newly diagnosed systemic lupus erythematosus

Concomitant presentation of thrombotic thrombocytopenic purpura, immune thrombocytopenia, and autoimmune hemolytic anemia in a patient with newly diagnosed systemic lupus erythematosus

Concurrence of immune thrombocytopenic purpura and thrombotic thrombocytopenic purpura: a case report and review of the literature

A case of systemic lupus erythematosus having concurrent Evans syndrome and acquired thrombotic thrombocytopenic purpura

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