# Atypical Presentation of Thrombotic Thrombocytopenic Purpura (TTP) as an Initial Manifestation of Systemic Lupus Erythematosus (SLE) in a Young Male

**Authors:** Kristen Pitts, Andres D Parga, Damian Casadesus, Stephen Symes

PMC · DOI: 10.7759/cureus.67567 · Cureus · 2024-08-23

## TL;DR

A young man with no prior health issues was diagnosed with TTP, which later revealed an underlying SLE, highlighting a rare initial presentation of the disease.

## Contribution

This case report highlights the rare initial manifestation of SLE as TTP in a young male with no prior medical history.

## Key findings

- The patient presented with altered mental status, headache, and right-sided numbness, leading to a TTP diagnosis.
- Further evaluation revealed the presence of SLE, indicating a rare initial manifestation of the disease.
- The case underscores the need for a comprehensive diagnostic approach when TTP is suspected.

## Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare but life-threatening hematologic disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, neurologic symptoms, renal impairment, and fever. The etiology of TTP often involves a severe deficiency in ADAMTS13 activity, resulting in the accumulation of ultra-large von Willebrand factor multimers and subsequent microvascular thrombosis. Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that can affect multiple organ systems, and although the initial presentation of SLE with TTP is rare, it necessitates a comprehensive diagnostic and therapeutic approach. We present a case of a 27-year-old male with no significant past medical history who developed altered mental status, headache, and right-sided numbness, leading to the diagnosis of TTP and subsequent detection of SLE.

## Linked entities

- **Proteins:** ADAMTS13 (ADAM metallopeptidase with thrombospondin type 1 motif 13)
- **Diseases:** Thrombotic Thrombocytopenic Purpura (MONDO:0018896), Systemic Lupus Erythematosus (MONDO:0007915)

## Full-text entities

- **Genes:** ADAMTS13 (ADAM metallopeptidase with thrombospondin type 1 motif 13) [NCBI Gene 11093] {aka ADAM-TS13, ADAMTS-13, C9orf8, VWFCP, vWF-CP}, VWF (von Willebrand factor) [NCBI Gene 7450] {aka F8VWF, VWD}
- **Diseases:** numbness (MESH:D006987), autoimmune disease (MESH:D001327), microvascular thrombosis (MESH:D017566), fever (MESH:D005334), headache (MESH:D006261), microangiopathic hemolytic anemia (MESH:D000743), hematologic disorder (MESH:D006402), thrombocytopenia (MESH:D013921), TTP (MESH:D011697), SLE (MESH:D008180), neurologic symptoms (MESH:D009461), renal impairment (MESH:D007674)

## Full text

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## References

8 references — full list in the complete paper: https://tomesphere.com/paper/PMC11342290/full.md

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Source: https://tomesphere.com/paper/PMC11342290