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Old 06-18-2012
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Default A 30-year-old woman reports becoming increasingly...

A 30-year-old woman reports becoming increasingly tired for the past 5 months. On physical examination, she is afebrile and has mild splenomegaly. Laboratory studies show a hemoglobin concentration of 11.8 mg/dl and Hct of 35.1%. The peripheral blood smear shows spherocytes and rare nucleated RBCs. The direct and indirect Coombs test results are positive at 37 C, although not at 4 C. Which of the following underlying diseases is most likely to be diagnosed in this patient?

A) Infectious mononucleosis
B) Mycoplasma pneumoniae infection
C) Hereditary spherocytosis
D) Escherichia coli septicemia
E) Systemic lupus erythematosus

Please try to explain your choice
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E) Systemic lupus erythematosus.... ?
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Originally Posted by Casandra View Post
A 30-year-old woman reports becoming increasingly tired for the past 5 months. On physical examination, she is afebrile and has mild splenomegaly. Laboratory studies show a hemoglobin concentration of 11.8 mg/dl and Hct of 35.1%. The peripheral blood smear shows spherocytes and rare nucleated RBCs. The direct and indirect Coombs test results are positive at 37 C, although not at 4 C. Which of the following underlying diseases is most likely to be diagnosed in this patient?

A) Infectious mononucleosis
B) Mycoplasma pneumoniae infection
C) Hereditary spherocytosis
D) Escherichia coli septicemia
E) Systemic lupus erythematosus

Please try to explain your choice
warm hemolytic anemia
extravascular hemolysis producing spherocytes
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Originally Posted by MedicalExaminer View Post
warm hemolytic anemia
extravascular hemolysis producing spherocytes
I was initially confused between C or E coz I didn't know of the warm hemolytic anemia! Googled about it though and Wikipedia is a good source. "Secondary to another disease, such as an antecedent upper respiratory tract infection, systemic lupus erythematosus or rheumatoid arthritis or a malignancy, such as chronic lymphocytic leukemia (CLL)". Could you explain how does it produce spherocytes? Isn't that a defect in the structural proteins ?
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Default correct answer :)

Quote:
Originally Posted by Hope2Pass View Post
I was initially confused between C or E coz I didn't know of the warm hemolytic anemia! Googled about it though and Wikipedia is a good source. "Secondary to another disease, such as an antecedent upper respiratory tract infection, systemic lupus erythematosus or rheumatoid arthritis or a malignancy, such as chronic lymphocytic leukemia (CLL)". Could you explain how does it produce spherocytes? Isn't that a defect in the structural proteins ?
Ok, I usually wait about 24 hrs to give the correct answer so that other people can solve it without the spoiler but this question is really interesting at least to me

correct answer is E) Systemic lupus erythematosus

This patient has warm autoimmune hemolytic anemia - secondary to SLE. Both Coombs tests confirmed presence of of anti-RBC antibodies and RBC coated with anti-RBC antibodies. Immunoglobulin that coats RBC is like opsonin and promotes splenic phagocytosis.

@Hope2Pass - you are right, spherocytes usually refer to hereditary spherocytosis that is caused by a molecular defect in one (or more) of the proteins of RBC cytoskeleton. BUT it can also be a result of autoimmune hemolytic anemia -> this presumably occurs during partial phagocytosis stimulated by antibodies on the cell surface. Only some parts (=proteins) of the RBC membrane are being destroyed so RBCs are being deprived of their biconcave shape, thus forming spherocytes.
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Originally Posted by Hope2Pass View Post
I was initially confused between C or E coz I didn't know of the warm hemolytic anemia! Googled about it though and Wikipedia is a good source. "Secondary to another disease, such as an antecedent upper respiratory tract infection, systemic lupus erythematosus or rheumatoid arthritis or a malignancy, such as chronic lymphocytic leukemia (CLL)". Could you explain how does it produce spherocytes? Isn't that a defect in the structural proteins ?
IgG coated RBCs are bitten by spleenic macrophages and lose some of their membrane , reduction in membrane outstreches the remaining part causing loss of biconcave shape producing spherocytes
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Yup answer is e...warm agglutinin...igG mediated...seen in cll and sle

Cold agglutinin...igM...seen in inf. mono and mycoplasma
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