Question #12 - klebsiella

[ArchivalUser]

A 21-year-old man with no significant past medical history presents to office with complaints of blood in his urine and mucosal bleeding while brushing his teeth. The patient complains of intermittent "ringing in the ears." He denies any drug or alcohol use. He has no family history of bleeding disorders. Petechiae are noted in the oral cavity, as is dried blood in the nostrils.

Laboratory studies show the following:

Hematocrit 32%; white blood cell count 8,000/mm3 with 60% neutrophils; platelet count 13,000; PT 13 seconds; PTT 28 seconds; LDH 1,200 U/L; elevated indirect bilirubin.

Coombs' test is positive; abdominal examination is normal; and the peripheral smear shows spherocytes.

What is the most likely diagnosis?

(A) Alport's syndrome
(B) Bernard-Soulier syndrome
© Felty's syndrome
(D) Thrombotic thrombocytopenic purpura
(E) Evans' syndrome
(F) Idiopathic thrombocytopenic purpura (ITP

[ArchivalUser]

E) Evans' syndrome

[ArchivalUser]

E??? these questions are hard!!

[ArchivalUser]

E) Evans' syndrome

Explanation:

Evans' syndrome is the association of autoimmune hemolysis with autoimmune thrombocytopenia. It is treated initially with steroids and may occasionally need splenectomy to control the disease.

Alport's syndrome is the congenital association of glomerulonephritis with sensorineural hearing loss and ocular problems.

Bernard-Soulier syndrome is a functional platelet disorder presenting with platelet-related bleeding with a normal platelet count.

Felty's syndrome is the association of rheumatoid arthritis with neutropenia and splenomegaly. It is occasionally associated with thrombocytopenia. This patient has no history of rheumatoid arthritis, and the spleen and neutrophil count are normal.

ITP would not give the evidence of hemolysis that is present here, such as an increased bilirubin, positive Coombs' test, high LDH, or anemia. This patient does not have the renal failure or fever associated with TTP. In addition, TTP should give fragmented red cells on peripheral smear.