Limits...
Microangiopathic Hemolytic Anemia in 57-year-old Woman with Borderline Serous Tumor of the Ovary: Real-Time Management of Common Pathways of Hemostatic Failure.

Morris GJ, Yaeger HC, Hamm F, Irwin S, Scialla SJ - Mediterr J Hematol Infect Dis (2012)

View Article: PubMed Central - PubMed

Affiliation: Mount Sinai Hospital of Queens, Long Island City, NY;

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

A 57-year-old female with history of myocardial infarction and need for cardiac catheterization with stent placement requiring clopidogrel for many years, as well as a history of hypertension requiring lisinopril, was evaluated by her gynecologist for ongoing pelvic pain... Pre-operative CA-125 level was normal, as was her complete blood count, hepatic, and renal function... Upon laparoscopy, there was found to be enlargement of the right ovary with excrescences, grossly suspicious for malignancy, thus requiring exploratory laparotomy... The differential diagnosis included microangiopathic hemolytic anemia (MAHA) associated with thrombotic thrombocytopenic purpura (TTP)/hemolytic-uremic syndrome (HUS), systemic inflammatory response syndrome (SIRS), antiphospholipid antibody syndrome, a compensated disseminated intravascular coagulation (DIC), and /or hypertension-associated MAHA... MAHA may be observed in patients who experience sepsis, disseminated carcinomatosis, disseminated intravascular coagulation, catastrophic antiphospholipid antibody syndrome (APS), organ transplantation, complications of pregnancy, malignant hypertension, and exposure to venoms, toxins, or antineoplastic agents such a mitomycin-C or cyclosporine. – Clopidogrel has previously been reported to cause TTP but usually this has been reported to occur within the first 2 weeks of initiation of the treatment., Typically the classical pentad of symptoms associated with TTP are fevers, central nervous systems changes, hemolytic anemia with shistocytes on a peripheral blood smear and associated with elevated LDH, renal insufficiency, and thrombocytopenia... Clinically, only a triad of the latter three are sufficient for a clinical diagnosis, which is curable with aggressive plasma exchange and pheresis. – Deficiency of ADAMTS13 von Willebrand factor-cleaving metalloprotease has been implicated in the pathogenesis of acute recurrent TTP, probably resulting from the combination of this deficiency due to autosomal recessive trait with decrease synthesis, intersecting with the mechanism of endothelial cell damage... Hence, when faced with these clinical signs, how does the consultant sort them out? We report another confounding trigger to the hemolytic cascade presented here... We present here a case of a 57-year-old woman previously on clopidogrel, who experienced MAHA after surgery for an ovarian mass which was deemed pathologically as a borderline serous tumor... However, post-operatively she experience fevers, renal insufficiency, hypertension, and MAHA with shistocytes and elevated LDH as well as thrombocytopenia, all suspicious for TTP, but further sorting of clinical information led to other differential diagnoses, including fever from atelectasis or SIRS, with elevated fibrinogen as in acute phase reaction; renal insufficiency from hypovolemia, as this responded to fluids; the possibility of malignant hypertension; and the possibility of DIC triggered from the release of intracellular contents from a necrotic ovarian tumor... Some common characteristics may include the activation of the coagulation system from direct contact of tumor cells with tissue factor, vis a vis, with elevated and qualitative Factor VIII, MAHA from fibrin stranding, or the presence of antiphospholipid antibodies... The difficult task is embarking on the correct and logical hematologic intervention(s), whether anticoagulation, steroids, transfusion, or aggressive plasma exchange; while life-saving in HUS/TTP, the latter may pose cardiovascular risk in some patients significant enough to consider the short-term risk versus benefit ratio... In, we summarize and propose a chronologic and strategic approach for the clinician who is faced with this dilemma of post-operative MAHA with thrombocytopenia and renal insufficiency with a large differential diagnosis including TTP, HIT, APL, and DIC. (a) Careful history must be taken with attention the onset of change in the CBC, induction of hemolysis, and consumption of platelets, concomitant with drug exposure, and put into clinical context; abrupt onset may suggest acute change, and a list of drugs may point toward more specific inciting mechanisms. (b) Physical examination with attention to hematologic manifestations such as purpura, thrombosis, and hemorrhage may help to distinguish the severity of the condition and would guide toward the absolute need for anticoagulation. (c) Immediate laboratory investigation of coagulation profile to distinguish DIC from other hemostatic disorders, including APL with the elevation of PTT alone and presence of associated antibodies, or a more normal coagulation profile in which platelet consumption may preside. (d) Visual assessment of blood cell morphology for red cell fragmentation or platelet aggregation is essential... The above described “markers” of impending hemostatic failure can help to determine which entity in the differential diagnosis to more fully pursue, and which specific intervention to follow, including plasmapheresis for TTP; anticoagulation for APL or even DIC (or its alternative for HIT); or treatment of the underlying cause with support from indicated transfusions for DIC... In this particular situation, a SIRS-like phenomenon was felt to prevail, and did not require a more intense intervention, but a successful supportive approach.

No MeSH data available.


Related in: MedlinePlus

Flow diagram of proposed analytical decision-making in cases of hemostatic failure, which stem from multiple possibilities in a differential diagnosis; while these converge at a common pathway marked by overt hemostatic failure, pursuit of a final diagnosis guides specific intervention. Abbreviations: Abs=antibodies; APL= antiphospholipid antibody syndrome; DIC=disseminated intravascular coagulation; FDP=fibrin degradation products; MAHA=microangiopathic hemolytic anemia; HIT = heparin-induced thrombocytopenia; plt=platelet; TTP= thrombotic thrombocytopenic purpura.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC3375672&req=5

f3-mjhid-4-1-e2012030: Flow diagram of proposed analytical decision-making in cases of hemostatic failure, which stem from multiple possibilities in a differential diagnosis; while these converge at a common pathway marked by overt hemostatic failure, pursuit of a final diagnosis guides specific intervention. Abbreviations: Abs=antibodies; APL= antiphospholipid antibody syndrome; DIC=disseminated intravascular coagulation; FDP=fibrin degradation products; MAHA=microangiopathic hemolytic anemia; HIT = heparin-induced thrombocytopenia; plt=platelet; TTP= thrombotic thrombocytopenic purpura.

Mentions: In Figure 3, we summarize and propose a chronologic and strategic approach for the clinician who is faced with this dilemma of post-operative MAHA with thrombocytopenia and renal insufficiency with a large differential diagnosis including TTP, HIT, APL, and DIC. (a) Careful history must be taken with attention the onset of change in the CBC, induction of hemolysis, and consumption of platelets, concomitant with drug exposure, and put into clinical context; abrupt onset may suggest acute change, and a list of drugs may point toward more specific inciting mechanisms. (b) Physical examination with attention to hematologic manifestations such as purpura, thrombosis, and hemorrhage may help to distinguish the severity of the condition and would guide toward the absolute need for anticoagulation. (c) Immediate laboratory investigation of coagulation profile to distinguish DIC from other hemostatic disorders, including APL with the elevation of PTT alone and presence of associated antibodies, or a more normal coagulation profile in which platelet consumption may preside. (d) Visual assessment of blood cell morphology for red cell fragmentation or platelet aggregation is essential. Further distillation of the pathogenesis of hemostatic failure is illustrated as the final ability of platelets to interact with larger blood vessels, and to form a clot with sustainable integrity. The above described “markers” of impending hemostatic failure can help to determine which entity in the differential diagnosis to more fully pursue, and which specific intervention to follow, including plasmapheresis for TTP; anticoagulation for APL or even DIC (or its alternative for HIT); or treatment of the underlying cause with support from indicated transfusions for DIC. In this particular situation, a SIRS-like phenomenon was felt to prevail, and did not require a more intense intervention, but a successful supportive approach.


Microangiopathic Hemolytic Anemia in 57-year-old Woman with Borderline Serous Tumor of the Ovary: Real-Time Management of Common Pathways of Hemostatic Failure.

Morris GJ, Yaeger HC, Hamm F, Irwin S, Scialla SJ - Mediterr J Hematol Infect Dis (2012)

Flow diagram of proposed analytical decision-making in cases of hemostatic failure, which stem from multiple possibilities in a differential diagnosis; while these converge at a common pathway marked by overt hemostatic failure, pursuit of a final diagnosis guides specific intervention. Abbreviations: Abs=antibodies; APL= antiphospholipid antibody syndrome; DIC=disseminated intravascular coagulation; FDP=fibrin degradation products; MAHA=microangiopathic hemolytic anemia; HIT = heparin-induced thrombocytopenia; plt=platelet; TTP= thrombotic thrombocytopenic purpura.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3375672&req=5

f3-mjhid-4-1-e2012030: Flow diagram of proposed analytical decision-making in cases of hemostatic failure, which stem from multiple possibilities in a differential diagnosis; while these converge at a common pathway marked by overt hemostatic failure, pursuit of a final diagnosis guides specific intervention. Abbreviations: Abs=antibodies; APL= antiphospholipid antibody syndrome; DIC=disseminated intravascular coagulation; FDP=fibrin degradation products; MAHA=microangiopathic hemolytic anemia; HIT = heparin-induced thrombocytopenia; plt=platelet; TTP= thrombotic thrombocytopenic purpura.
Mentions: In Figure 3, we summarize and propose a chronologic and strategic approach for the clinician who is faced with this dilemma of post-operative MAHA with thrombocytopenia and renal insufficiency with a large differential diagnosis including TTP, HIT, APL, and DIC. (a) Careful history must be taken with attention the onset of change in the CBC, induction of hemolysis, and consumption of platelets, concomitant with drug exposure, and put into clinical context; abrupt onset may suggest acute change, and a list of drugs may point toward more specific inciting mechanisms. (b) Physical examination with attention to hematologic manifestations such as purpura, thrombosis, and hemorrhage may help to distinguish the severity of the condition and would guide toward the absolute need for anticoagulation. (c) Immediate laboratory investigation of coagulation profile to distinguish DIC from other hemostatic disorders, including APL with the elevation of PTT alone and presence of associated antibodies, or a more normal coagulation profile in which platelet consumption may preside. (d) Visual assessment of blood cell morphology for red cell fragmentation or platelet aggregation is essential. Further distillation of the pathogenesis of hemostatic failure is illustrated as the final ability of platelets to interact with larger blood vessels, and to form a clot with sustainable integrity. The above described “markers” of impending hemostatic failure can help to determine which entity in the differential diagnosis to more fully pursue, and which specific intervention to follow, including plasmapheresis for TTP; anticoagulation for APL or even DIC (or its alternative for HIT); or treatment of the underlying cause with support from indicated transfusions for DIC. In this particular situation, a SIRS-like phenomenon was felt to prevail, and did not require a more intense intervention, but a successful supportive approach.

View Article: PubMed Central - PubMed

Affiliation: Mount Sinai Hospital of Queens, Long Island City, NY;

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

A 57-year-old female with history of myocardial infarction and need for cardiac catheterization with stent placement requiring clopidogrel for many years, as well as a history of hypertension requiring lisinopril, was evaluated by her gynecologist for ongoing pelvic pain... Pre-operative CA-125 level was normal, as was her complete blood count, hepatic, and renal function... Upon laparoscopy, there was found to be enlargement of the right ovary with excrescences, grossly suspicious for malignancy, thus requiring exploratory laparotomy... The differential diagnosis included microangiopathic hemolytic anemia (MAHA) associated with thrombotic thrombocytopenic purpura (TTP)/hemolytic-uremic syndrome (HUS), systemic inflammatory response syndrome (SIRS), antiphospholipid antibody syndrome, a compensated disseminated intravascular coagulation (DIC), and /or hypertension-associated MAHA... MAHA may be observed in patients who experience sepsis, disseminated carcinomatosis, disseminated intravascular coagulation, catastrophic antiphospholipid antibody syndrome (APS), organ transplantation, complications of pregnancy, malignant hypertension, and exposure to venoms, toxins, or antineoplastic agents such a mitomycin-C or cyclosporine. – Clopidogrel has previously been reported to cause TTP but usually this has been reported to occur within the first 2 weeks of initiation of the treatment., Typically the classical pentad of symptoms associated with TTP are fevers, central nervous systems changes, hemolytic anemia with shistocytes on a peripheral blood smear and associated with elevated LDH, renal insufficiency, and thrombocytopenia... Clinically, only a triad of the latter three are sufficient for a clinical diagnosis, which is curable with aggressive plasma exchange and pheresis. – Deficiency of ADAMTS13 von Willebrand factor-cleaving metalloprotease has been implicated in the pathogenesis of acute recurrent TTP, probably resulting from the combination of this deficiency due to autosomal recessive trait with decrease synthesis, intersecting with the mechanism of endothelial cell damage... Hence, when faced with these clinical signs, how does the consultant sort them out? We report another confounding trigger to the hemolytic cascade presented here... We present here a case of a 57-year-old woman previously on clopidogrel, who experienced MAHA after surgery for an ovarian mass which was deemed pathologically as a borderline serous tumor... However, post-operatively she experience fevers, renal insufficiency, hypertension, and MAHA with shistocytes and elevated LDH as well as thrombocytopenia, all suspicious for TTP, but further sorting of clinical information led to other differential diagnoses, including fever from atelectasis or SIRS, with elevated fibrinogen as in acute phase reaction; renal insufficiency from hypovolemia, as this responded to fluids; the possibility of malignant hypertension; and the possibility of DIC triggered from the release of intracellular contents from a necrotic ovarian tumor... Some common characteristics may include the activation of the coagulation system from direct contact of tumor cells with tissue factor, vis a vis, with elevated and qualitative Factor VIII, MAHA from fibrin stranding, or the presence of antiphospholipid antibodies... The difficult task is embarking on the correct and logical hematologic intervention(s), whether anticoagulation, steroids, transfusion, or aggressive plasma exchange; while life-saving in HUS/TTP, the latter may pose cardiovascular risk in some patients significant enough to consider the short-term risk versus benefit ratio... In, we summarize and propose a chronologic and strategic approach for the clinician who is faced with this dilemma of post-operative MAHA with thrombocytopenia and renal insufficiency with a large differential diagnosis including TTP, HIT, APL, and DIC. (a) Careful history must be taken with attention the onset of change in the CBC, induction of hemolysis, and consumption of platelets, concomitant with drug exposure, and put into clinical context; abrupt onset may suggest acute change, and a list of drugs may point toward more specific inciting mechanisms. (b) Physical examination with attention to hematologic manifestations such as purpura, thrombosis, and hemorrhage may help to distinguish the severity of the condition and would guide toward the absolute need for anticoagulation. (c) Immediate laboratory investigation of coagulation profile to distinguish DIC from other hemostatic disorders, including APL with the elevation of PTT alone and presence of associated antibodies, or a more normal coagulation profile in which platelet consumption may preside. (d) Visual assessment of blood cell morphology for red cell fragmentation or platelet aggregation is essential... The above described “markers” of impending hemostatic failure can help to determine which entity in the differential diagnosis to more fully pursue, and which specific intervention to follow, including plasmapheresis for TTP; anticoagulation for APL or even DIC (or its alternative for HIT); or treatment of the underlying cause with support from indicated transfusions for DIC... In this particular situation, a SIRS-like phenomenon was felt to prevail, and did not require a more intense intervention, but a successful supportive approach.

No MeSH data available.


Related in: MedlinePlus