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Renal thrombotic microangiopathy in a patient with septic disseminated intravascular coagulation.

Sakamaki Y, Konishi K, Hayashi K, Hashiguchi A, Hayashi M, Kubota E, Saruta T, Itoh H - BMC Nephrol (2013)

Bottom Line: We therefore suspected the presence of TMA and started plasma exchange, which resulted in an impressive improvement in consciousness as well as the laboratory abnormalities.The follow-up studies performed after the successful treatment of TMA showed that her plasma ADAMTS-13 activity level remained persistently low.It is surmised that septic DIC occurring in the presence of preexisting reduced ADAMTS-13 activity have led to the development of secondary TMA in the present case.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Internal Medicine, Shizuoka Red Cross Hospital, 8-2 Otemachi, Aoi-Ku, Shizuoka-City, Shizuoka 420-0853, Japan. yusuke0225@dance.ocn.ne.jp.

ABSTRACT

Background: The mechanism for the development of thrombotic microangiopathy (TMA) during sepsis has only been partially elucidated. TMA is recognized as a disease caused by various factors, and may be involved in the emergence of organ damage in severe sepsis. Here we report a case of TMA that followed disseminated intravascular coagulation (DIC) due to severe infection in a patient with a reduced ADAMTS-13 activity level.

Case presentation: An 86-year-old Japanese woman was admitted to our hospital because of low back pain and fever. A careful evaluation led to a diagnosis of acute obstructive pyelonephritis due to a ureteral stone. Proteus mirabilis was isolated from both blood and urine cultures. The patient developed systemic inflammatory response syndrome and DIC, and was treated with antibiotics and daily continuous hemodiafiltration. Although infection and the coagulation abnormalities due to DIC were successfully controlled, renal failure persisted and her consciousness level deteriorated progressively in association with severe thrombocytopenia and microangiopathic hemolytic anemia. We therefore suspected the presence of TMA and started plasma exchange, which resulted in an impressive improvement in consciousness as well as the laboratory abnormalities. The ADAMTS-13 activity was 44% and the patient tested negative for the ADAMTS-13 inhibitor prior to the initiation of plasma exchange. A renal biopsy was performed to determine the etiology of acute renal injury, which revealed findings that were interpreted to be compatible with the sequelae of TMA. The follow-up studies performed after the successful treatment of TMA showed that her plasma ADAMTS-13 activity level remained persistently low. It is surmised that septic DIC occurring in the presence of preexisting reduced ADAMTS-13 activity have led to the development of secondary TMA in the present case.

Conclusion: The present case suggests that TMA can be superimposed on sepsis-induced DIC, and plasma exchange is expected to be beneficial in such situations. Clinicians should consider the possibility of secondary TMA that follows sepsis-induced DIC in certain indicative clinical settings.

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Histopathological findings of renal biopsy. (A) Light photomicrograph showing two glomeruli with widened capillary lumina containing red blood cells (Hematoxylin and eosin stain; original magnification, ×200). (B) Severe tubular necrosis with a loss of cellular detail (Hematoxylin and eosin stain; original magnification, ×400). (C, D) Serial sections of an afferent arteriole with obliterative intimal change (C; arrowhead) and intraluminal thrombus formation (D; arrow) (Periodic acid silver methenamin stain; original magnification, ×400). (E) Smaller interlobular arteries and arterioles showed occlusion or extensive narrowing of their lumen which was interpreted to represent the sequel of TMA (D; Periodic acid-Schiff stain; original magnification, ×400).
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Figure 1: Histopathological findings of renal biopsy. (A) Light photomicrograph showing two glomeruli with widened capillary lumina containing red blood cells (Hematoxylin and eosin stain; original magnification, ×200). (B) Severe tubular necrosis with a loss of cellular detail (Hematoxylin and eosin stain; original magnification, ×400). (C, D) Serial sections of an afferent arteriole with obliterative intimal change (C; arrowhead) and intraluminal thrombus formation (D; arrow) (Periodic acid silver methenamin stain; original magnification, ×400). (E) Smaller interlobular arteries and arterioles showed occlusion or extensive narrowing of their lumen which was interpreted to represent the sequel of TMA (D; Periodic acid-Schiff stain; original magnification, ×400).

Mentions: To determine the etiology of the renal disease which was unresponsive to plasma exchange, renal biopsy was performed on day 26. The specimen for light microscopy contained 15 glomeruli; 7 exhibited global sclerosis, 2 severe segmental sclerosis, and 2 exhibited only minor abnormalities. The remaining 4 glomeruli had dilated capillary loops that were filled with red blood cells (Figure 1-A). Aggregations of severe tubular necrosis were seen in several foci (Figure 1-B). One of these glomeruli had an afferent arteriole with occlusive intimal changes (Figure 1-C; arrowhead) and what appeared to be a small intraluminal thrombus (Figure 1-D; arrow). Arteriosclerotic fibrous thickening of the intima was seen in the larger arteries. Smaller interlobular arteries and arterioles, on the other hand, showed occlusion or extensive narrowing of their lumen which was interpreted to represent the sequel of TMA (Figure 1-E). Fibrinoid necrosis of the vessel wall was not observed. No obvious thrombi were observed in the vascular lumina except for the above-mentioned modest lesion in an afferent arteriole. Tubular atrophy and interstitial fibrosis were seen in approximately 50% of the cortical area. Immunofluorescence studies showed no immunoglobulin, complement or fibrinogen deposition in the glomeruli or the blood vessels.


Renal thrombotic microangiopathy in a patient with septic disseminated intravascular coagulation.

Sakamaki Y, Konishi K, Hayashi K, Hashiguchi A, Hayashi M, Kubota E, Saruta T, Itoh H - BMC Nephrol (2013)

Histopathological findings of renal biopsy. (A) Light photomicrograph showing two glomeruli with widened capillary lumina containing red blood cells (Hematoxylin and eosin stain; original magnification, ×200). (B) Severe tubular necrosis with a loss of cellular detail (Hematoxylin and eosin stain; original magnification, ×400). (C, D) Serial sections of an afferent arteriole with obliterative intimal change (C; arrowhead) and intraluminal thrombus formation (D; arrow) (Periodic acid silver methenamin stain; original magnification, ×400). (E) Smaller interlobular arteries and arterioles showed occlusion or extensive narrowing of their lumen which was interpreted to represent the sequel of TMA (D; Periodic acid-Schiff stain; original magnification, ×400).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4222681&req=5

Figure 1: Histopathological findings of renal biopsy. (A) Light photomicrograph showing two glomeruli with widened capillary lumina containing red blood cells (Hematoxylin and eosin stain; original magnification, ×200). (B) Severe tubular necrosis with a loss of cellular detail (Hematoxylin and eosin stain; original magnification, ×400). (C, D) Serial sections of an afferent arteriole with obliterative intimal change (C; arrowhead) and intraluminal thrombus formation (D; arrow) (Periodic acid silver methenamin stain; original magnification, ×400). (E) Smaller interlobular arteries and arterioles showed occlusion or extensive narrowing of their lumen which was interpreted to represent the sequel of TMA (D; Periodic acid-Schiff stain; original magnification, ×400).
Mentions: To determine the etiology of the renal disease which was unresponsive to plasma exchange, renal biopsy was performed on day 26. The specimen for light microscopy contained 15 glomeruli; 7 exhibited global sclerosis, 2 severe segmental sclerosis, and 2 exhibited only minor abnormalities. The remaining 4 glomeruli had dilated capillary loops that were filled with red blood cells (Figure 1-A). Aggregations of severe tubular necrosis were seen in several foci (Figure 1-B). One of these glomeruli had an afferent arteriole with occlusive intimal changes (Figure 1-C; arrowhead) and what appeared to be a small intraluminal thrombus (Figure 1-D; arrow). Arteriosclerotic fibrous thickening of the intima was seen in the larger arteries. Smaller interlobular arteries and arterioles, on the other hand, showed occlusion or extensive narrowing of their lumen which was interpreted to represent the sequel of TMA (Figure 1-E). Fibrinoid necrosis of the vessel wall was not observed. No obvious thrombi were observed in the vascular lumina except for the above-mentioned modest lesion in an afferent arteriole. Tubular atrophy and interstitial fibrosis were seen in approximately 50% of the cortical area. Immunofluorescence studies showed no immunoglobulin, complement or fibrinogen deposition in the glomeruli or the blood vessels.

Bottom Line: We therefore suspected the presence of TMA and started plasma exchange, which resulted in an impressive improvement in consciousness as well as the laboratory abnormalities.The follow-up studies performed after the successful treatment of TMA showed that her plasma ADAMTS-13 activity level remained persistently low.It is surmised that septic DIC occurring in the presence of preexisting reduced ADAMTS-13 activity have led to the development of secondary TMA in the present case.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Internal Medicine, Shizuoka Red Cross Hospital, 8-2 Otemachi, Aoi-Ku, Shizuoka-City, Shizuoka 420-0853, Japan. yusuke0225@dance.ocn.ne.jp.

ABSTRACT

Background: The mechanism for the development of thrombotic microangiopathy (TMA) during sepsis has only been partially elucidated. TMA is recognized as a disease caused by various factors, and may be involved in the emergence of organ damage in severe sepsis. Here we report a case of TMA that followed disseminated intravascular coagulation (DIC) due to severe infection in a patient with a reduced ADAMTS-13 activity level.

Case presentation: An 86-year-old Japanese woman was admitted to our hospital because of low back pain and fever. A careful evaluation led to a diagnosis of acute obstructive pyelonephritis due to a ureteral stone. Proteus mirabilis was isolated from both blood and urine cultures. The patient developed systemic inflammatory response syndrome and DIC, and was treated with antibiotics and daily continuous hemodiafiltration. Although infection and the coagulation abnormalities due to DIC were successfully controlled, renal failure persisted and her consciousness level deteriorated progressively in association with severe thrombocytopenia and microangiopathic hemolytic anemia. We therefore suspected the presence of TMA and started plasma exchange, which resulted in an impressive improvement in consciousness as well as the laboratory abnormalities. The ADAMTS-13 activity was 44% and the patient tested negative for the ADAMTS-13 inhibitor prior to the initiation of plasma exchange. A renal biopsy was performed to determine the etiology of acute renal injury, which revealed findings that were interpreted to be compatible with the sequelae of TMA. The follow-up studies performed after the successful treatment of TMA showed that her plasma ADAMTS-13 activity level remained persistently low. It is surmised that septic DIC occurring in the presence of preexisting reduced ADAMTS-13 activity have led to the development of secondary TMA in the present case.

Conclusion: The present case suggests that TMA can be superimposed on sepsis-induced DIC, and plasma exchange is expected to be beneficial in such situations. Clinicians should consider the possibility of secondary TMA that follows sepsis-induced DIC in certain indicative clinical settings.

Show MeSH
Related in: MedlinePlus