A Rare Diagnosis in the Post‐Haart Era

1Northshore‐Long Island Jewish, Manhasset, NY
2Hofstra North Shore‐LIJ Health System, Manhasset, NY

Meeting: Hospital Medicine 2014, March 24-27, Las Vegas, Nev.

Abstract number: 292

Case Presentation:

A 57 year old man with AIDS (CD4=27) was seen at this hospital because of progressive fatigue. He became less active for the past 3 weeks due to gradually worsening fatigue. This progressed to where the patient could not walk on his own and was feeling confused.

In the ED the patient denied any history of fever or chills, headache, neck stiffness, myalgias. He reported non‐adherence with his anti‐retroviral medication. While in the ED the patient had a grand mal seizure. He was given keppra and fosphenytoin. Physical exam was noncontributory. He had a lactate of 14.6, creatine kinase of 339 and BUN:Cr of 128:9.63. Empiric antibiotics for meningitis were administered. A head CT revealed an area of white matter lucency in the posterior left frontal lobe. A repeat CBC showed a hemoglobin of 7.9 (admission Hb = 11) and a platelet level of 9000 (admission = 67, 000). A blood smear showed schistocytes. LDH was markedly elevated at 2308, haptoglobin <20. Given the patient’s renal failure, neurological status, hemolytic anemia and thrombocytopenia, the patient was started on plasmapheresis for presumed thrombotic thrombocytopenic purpra (TTP). Hemodialysis was initiated. He was diagnosed with HIV associated thrombotic microangiopathic anemia (TMA).

Discussion:

TMA represents a pathological entity defined by thrombosis in capillaries and arterioles due to endothelial damage, which exposes the subendothelial lining, activating the coagulation cascade and causing platelet aggregation. TMA was first described in an AIDS patient by Boccia et al in 1984.

The two prototypical diseases in this spectrum include hemolytic uremic syndrome (HUS) and TTP. HUS is more commonly seen in the pediatric population in those infected with E. coli O157:H7. TTP is characterized by a pentad of fever, hemolytic anemia, thrombocytopenia, renal failure and neurological symptoms. While the incidence of idiopathic TMA in the general population in immunocompetent individuals is rare‐on the order of about 0.001%‐the incidence in HIV infected patients was determined to be significantly higher at 0.3%. The diagnosis is often difficult to make, given that patients rarely present with all of the findings of the classic pentad. Those with HIV will have the same clinical manifestations as immunocompetent patients with TMP. HIV infection itself causes thrombocytopenia, and those with end stage AIDS often have fever, neurologic symptoms or kidney disease.

Evidence for the treatment for HIV associated TMA is limited. Plasmapheresis with plasma exchange is the standard therapy, yet has shown inconsistent results. Corticosteroids, splenectomy, immunoglobulin infusions and antiplatelet agents have been used in HIV‐associated TMA with varying success. Treatment with HAART should also be initiated as soon as possible.

Conclusions:

Our patient’s noncompliance with his antiretroviral medication may have predisposed him to develop HIV‐associated TMA. In AIDS patients who present with thrombocytopenia and multiorgan failure this diagnosis should be considered as prompt recognition and early treatment can help reduce mortality. The patient’s platelet count gradually increased to over 100,000 after 9 sessions of plasma exchange and prednisone therapy, and the hemolysis resolved as well. While his mental status and platelet counts have improved back to baseline the patient will need to be on permanent hemodialysis.

To cite this abstract:

Abbasi M, Oks M, Kumar D. A Rare Diagnosis in the Post‐Haart Era. Abstract published at Hospital Medicine 2014, March 24-27, Las Vegas, Nev. Abstract 292. Journal of Hospital Medicine. 2014; 9 (suppl 2). https://www.shmabstracts.com/abstract/a-rare-diagnosis-in-the-posthaart-era/. Accessed March 31, 2020.

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