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Real Doctors (Life Makers)  |  Clinical  |  Medicine & medical subspecialities  |  A 35-year-old male with complaints of abdominal pain and fever « previous next »
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cleo_md
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A 35-year-old male with complaints of abdominal pain and fever
« on: /November/ 23, 2005, 03:59:08 PM »

A 35-year-old male with complaints of abdominal pain and fever? ?
 



A 35-year-old man presented to the emergency department with abdominal pain that had begun the day before and progressively worsened. He denied experiencing nausea, vomiting, or diarrhea. He also complained of a mild headache that had been present off and on for several days. He reported no medical problems and denied excessive alcohol intake or illicit drug use.

On examination, the patient's blood pressure was 121/64 mm Hg, and his pulse was 114 beats per minute. His temperature was 100.2?F. He had diffuse abdominal tenderness but no guarding. He was pale, but he had no petechiae or other rash.

The laboratory results suggested the diagnosis. He had a hemoglobin level of 11 g/dL and a platelet count of 39,600/?L, and schistocytes were reported on the peripheral smear. His serum lactate dehydrogenase (LDH) level was markedly elevated at 1,540 U/L. The patient's bleeding times were normal, the remainder of his liver enzymes were at normal levels, his blood urea nitrogen measurement was 46 mg/dL, and his creatinine level was 1.6 mg/dL.

Based on these laboratory results, the patient was admitted to the hospital for treatment with plasmapheresis.

What should be included in the differential diagnosis for this patient?
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dr_b.e.s.m
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Re: A 35-year-old male with complaints of abdominal pain and fever
« Reply #1 on: /November/ 24, 2005, 05:12:36 PM »

What's this Dr Cleo? Is it TTP?

Fragmented RBCs = micro angiopathic haemolytic anemia. That's the diffrential diagnosis (TTP, DIC, HUS, artificial valve).

No history of valve replacement , coagulations studies are normal (so not DIC) , there is headache (neurological manifestations) so it's more likely TTP than HUS.

He has fever , abd. pain (I read it's due to pancreatitis! is it true? ) , mild headache (neuro c/o). Also the elevated BUN and creat. , low hb , shistocytes , and markedly elevated LDH that's out of proportion to haemolysis (hb isn't very low , it's 11 and LDH is more than 1000 ! ) .

thank you Dr Cleo for this case , waiting for your answer.? Smiley

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dr_b.e.s.m
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Re: A 35-year-old male with complaints of abdominal pain and fever
« Reply #2 on: /February/ 14, 2006, 09:25:45 AM »

Where is the answer of this case?  Embarrassed
 
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cleo_md
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Re: A 35-year-old male with complaints of abdominal pain and fever
« Reply #3 on: /February/ 14, 2006, 04:55:13 PM »

Dr BESM , So sorry , i posted the case , saw your answer one afternoon and felt good you knew the answer and totally forgot to comment! Silly me!

You are right

The initial presentation, abdominal pain and fever, is nonspecific and can be present in many problems. The findings of anemia, schistocytes, and an elevated LDH level point to a hemolytic process. Disseminated intravascular coagulation (DIC) is associated with thrombocytopenia, but with DIC the prothrombin time (PT) and partial thromboplastin time (PTT) would be abnormal. Idiopathic thrombocytopenic purpura is characterized by a low platelet count, but the hemoglobin and hematocrit counts are usually normal. Hemolytic uremic syndrome (HUS) usually occurs in children and presents with acute renal failure and mild thrombocytopenia. HELLP (hemolysis, elevated liver enzymes, low platelets) syndrome is a disorder in pregnancy that is associated with eclampsia.

The most likely diagnosis in this particular patient is thrombotic thrombocytopenic purpura (TTP) previously known as Moschowitz's syndrome. The classic presentation includes thrombocytopenia, microangiopathic hemolytic anemia, decreased renal function, diffuse nonfocal neurologic symptoms, and fever. This presentation is noted in about half of patients at the time of diagnosis.



What is the pathophysiology of TTP?

There are two forms of TTP: an acquired form and a familial form. The acquired form is characterized by an acute course and is more common in adults. This idiopathic form can be recurrent in 11% to 36% of patients. The familial form of TTP is more common in children and is characterized by a chronic relapsing course.

In the 1980s, it was discovered that patients with TTP had unusually large (UL, or ultralarge) multimers of von Willebrand factor (VWF). The multimers of VWF bind to glycoprotein receptors on platelets, inducing platelet adhesion and aggregation. A specific VWF-cleaving metalloprotease degrades the UL multimers, making them less efficient and allowing control of their contribution to the clotting process. Patients with TTP appear to be deficient in this enzyme, known as ADAMTS13, and the UL multimers are not cleaved (hence the large size). Subsequently, it was discovered that an immunoglobulin G autoantibody to the protease was active during episodes of idiopathic TTP, but not after recovery. Patients with the familial form of TTP have less than 5% of the normal ADAMTS13 levels. Treatment with plasmapheresis or fresh frozen plasma replaces the deficient enzyme, allowing normal degradation of the UL VWF.

More recent findings have suggested that although ADAMTS13 deficiency is a major risk factor for TTP, it is not always abnormal in patients with TTP, and some patients with abnormal levels of ADAMTS13 do not develop TTP.

Microvascular occlusion caused by excessive systemic platelet aggregation results in organ ischemia, thrombocytopenia, and hemolysis due to fragmentation of the erythrocytes (resulting in schistocytes) in areas of turbulence The hemolysis and local ischemic damage causes the release of LDH, and serum levels are typically very high. Renal failure, neurologic abnormalities, ischemic damage to the organs, and severe anemia occur as the disease progresses. Stroke, myocardial infarction, renal infarction, pancreatitis, and bowel infarction are some examples of tissue destruction due to intravascular thrombosis.

Up to one fourth of cases occur in women who are postpartum or pregnant, most commonly before 24 weeks of gestation. During pregnancy, TTP must be distinguished from HELLP syndrome. TTP is also more common in patients with cancer and HIV infection and has been reported in patients treated with some medications (such as ticlopidine and clopidogrel), patients with infections with Escherichia coli 0157:H7, and persons who have sustained bee stings or spider bites.

What physical and laboratory findings confirm the diagnosis?

Patients may present with nonspecific complaints such as fever and malaise. Thrombocytopenia and anemia with peripheral schistocytes and elevated LDH levels strongly suggest the diagnosis. Patients often present with altered mental status and may have nonfocal neurologic symptoms such as headaches, visual disturbances, and seizures or focal deficits due to ischemia. Heart failure, abdominal pain, renal impairment, hematuria, orthostatic hypotension, petechiae, purpura, jaundice, and splenomegaly may be found, although these are not always present.

The laboratory findings are as described but also are characteristic in that the PT and activated PTT are typically normal, as are fibrinogen levels and D-dimer levels. Urinalysis may show proteinuria and hematuria, and the creatinine level is often mildly elevated. Platelet levels are often below 20,000/?L.

Biopsy of the bone marrow or the gingiva may help confirm the diagnosis, as hyaline thrombi are present in up to half the cases. In addition, there is an increase in the marrow megakaryocytes.

There are no specific laboratory tests for TTP, but ADAMTS13 assays that measure plasma VWF multimer cleavage have been developed for experimental use. A recombinant substrate with residues of VWF have been devised that may allow the development of a simple assay that can be used in a clinical laboratory. These tests would be useful in determining the prognosis because patients with the idiopathic form of the disease and severely decreased levels of ADAMTS13 have the best response to plasmapheresis

What distinguishes TTP from HUS?

Both familial TTP and HUS can occur in children and are characterized by hemolysis, renal impairment, and thrombocytopenia. However, patients with HUS do not have a deficiency of ADAMTS13. Clinically, patients with HUS have more severe renal disease and do not usually respond to treatment with plasma exchange. HUS is associated with infection with E coli 0157:H7, but diarrhea may not be present. Thus, atypical HUS may be confused with TTP.


What treatments are effective for TTP?


Plasma exchange through plasmapheresis is effective in most patients and remains the cornerstone of treatment. Any antibodies to ADAMTS13 are removed and the protease deficiency is corrected. If plasmapheresis is not readily available, plasma infusion with fresh frozen plasma can be done as a temporizing measure. Up to 77% of patients respond to treatment with daily plasmapheresis with plasma exchange. Patients with the familial form of TTP can be treated with weekly infusions of fresh frozen plasma or cryosupernatant to replace ADAMTS13.

If there is an inadequate response to plasmapheresis (based on platelet counts), prednisone may offer some benefit. Splenectomy, immunosuppression (vincristine, azathioprine, cyclophosphamide), autologous bone marrow transplant, and staphylococcal protein A adsorption can be attempted in refractory cases. Platelet infusions in patients with TTP can be fatal because of increased platelet aggregation, so it is imperative to rule out TTP in a patient with suspected DIC (for whom platelet infusion would be indicated). A chimeric monoclonal antibody, rituximab, has also been used successfully in some cases. It appears to work by binding the anti-ADAMTS13 antibodies, allowing recovery of the protease activity.

In the future, gene therapy may be an option for treatment of patients with the familial form of TTP.

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3abeer
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Re: A 35-year-old male with complaints of abdominal pain and fever
« Reply #4 on: /February/ 15, 2006, 10:41:59 AM »

one thing more dr baha2
HUS is likely to occur in peds age group
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cleo_md
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Re: A 35-year-old male with complaints of abdominal pain and fever
« Reply #5 on: /February/ 16, 2006, 06:31:25 PM »

TTP - peripheral smear showing shistocytes

* TTP - peripheral smear.jpg (8.49 KB - downloaded 9 times.)
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cleo_md
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Re: A 35-year-old male with complaints of abdominal pain and fever
« Reply #6 on: /February/ 16, 2006, 06:32:57 PM »

And one last comment  - Why is TTP so important to know??

The mortality rate of TTP exceeds 90% without therapy
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