Ethics code: IR.AJUMS.REC.1403.552
Abstract: (29 Views)
A B S T R A C T
Background and Objectives
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy characterized by thrombocytopenia, hemolytic anemia, neurological symptoms, renal dysfunction, and fever. Although therapeutic plasma exchange (TPE) is the cornerstone of treatment management, responses can vary significantly among patients. This report describes an unusual recurrence of TTP in which platelet recovery was inconsistent with biochemical recovery, emphasizing the need for individualized treatment strategies.
Materials and Methods
A 38-year-old female patient with a 2-year history of TTP presented to Baqaei 2 Hospital in Ahvaz with presentations including altered level of consciousness, seizures, anemia, thrombocytopenia, and marked elevation of lactate dehydrogenase (LDH). At initial evaluation, the patient's platelet count was 25 ×109 /L, hemoglobin level was 7.5 g/dL, and LDH was 2300 units/L. Treatment with plasma exchange with corticosteroids was initiated, which resulted in initial improvement in platelet count and hemolysis indices. After six sessions of plasma exchange, the platelet count increased to more than 150×109/L on one occasion; however, a decrease to 83×109/L was observed in the next session. Despite continuing treatment for 12 sessions, the platelet count only reached 107×109 /L. After completing 18 sessions of daily plasma exchange, although the LDH level returned to the normal range, the platelet count still did not exceed 150×109 /L, indicating limited efficacy of additional plasma exchange sessions. Subsequently, plasma exchange was discontinued and corticosteroids were continued with the addition of rituximab (375 mg weekly for four weeks), resulting in a gradual and sustained increase in platelet count to the normal range.
Conclusions
This study demonstrates that hematologic improvement in TTP is not always accompanied by biochemical normalization. Persistent thrombocytopenia with normal LDH may indicate immune or endothelial factors that are not resolved by plasma exchange alone. Close monitoring, individual adjustments, and early initiation of immunotherapy such as rituximab are essential to optimize outcomes.
Background and Objectives
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening thrombotic microangiopathy characterized by thrombocytopenia, hemolytic anemia, neurological symptoms, renal dysfunction, and fever. Although therapeutic plasma exchange (TPE) is the cornerstone of treatment management, responses can vary significantly among patients. This report describes an unusual recurrence of TTP in which platelet recovery was inconsistent with biochemical recovery, emphasizing the need for individualized treatment strategies.
Materials and Methods
A 38-year-old female patient with a 2-year history of TTP presented to Baqaei 2 Hospital in Ahvaz with presentations including altered level of consciousness, seizures, anemia, thrombocytopenia, and marked elevation of lactate dehydrogenase (LDH). At initial evaluation, the patient's platelet count was 25 ×109 /L, hemoglobin level was 7.5 g/dL, and LDH was 2300 units/L. Treatment with plasma exchange with corticosteroids was initiated, which resulted in initial improvement in platelet count and hemolysis indices. After six sessions of plasma exchange, the platelet count increased to more than 150×109/L on one occasion; however, a decrease to 83×109/L was observed in the next session. Despite continuing treatment for 12 sessions, the platelet count only reached 107×109 /L. After completing 18 sessions of daily plasma exchange, although the LDH level returned to the normal range, the platelet count still did not exceed 150×109 /L, indicating limited efficacy of additional plasma exchange sessions. Subsequently, plasma exchange was discontinued and corticosteroids were continued with the addition of rituximab (375 mg weekly for four weeks), resulting in a gradual and sustained increase in platelet count to the normal range.
Conclusions
This study demonstrates that hematologic improvement in TTP is not always accompanied by biochemical normalization. Persistent thrombocytopenia with normal LDH may indicate immune or endothelial factors that are not resolved by plasma exchange alone. Close monitoring, individual adjustments, and early initiation of immunotherapy such as rituximab are essential to optimize outcomes.
Type of Study: Research |
Subject:
Hematology
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