**Question:** Liver biopsy in a patient with cirrhosis demonstrates markedly increased iron stores. The patient has no known history of hemolytic anemia or other potential causes of a secondary increase in hepatic iron stores. This patient's primary disorder is most strongly associated with which of the following HLA alleles?
A. HLA-B8
B. HLA-Dw2
C. HLA-Dw3
D. HLA-Dw4
**Answer Explanation:**
**Core Concept:** Liver biopsy in cirrhosis.
In patients with cirrhosis, liver biopsy can reveal increased iron stores, which is a feature of hemochromatosis, a primary liver disorder characterized by excessive iron absorption and deposition in the liver, leading to liver dysfunction and cirrhosis.
**Why the Correct Answer is Right:**
The correct answer is HLA-Dw2 because hemochromatosis is a genetic disorder that is strongly associated with HLA-Dw2. The other options (HLA-B8, HLA-Dw3, and HLA-Dw4) are also associated with hemochromatosis but are less commonly associated compared to HLA-Dw2.
**Why Each Wrong Option is Incorrect:**
A. HLA-B8: Although HLA-B8 is associated with hemochromatosis, it is less strongly linked compared to HLA-Dw2.
B. HLA-Dw2: Explained above that HLA-Dw2 is strongly associated with hemochromatosis.
C. HLA-Dw3: While HLA-Dw3 is also associated with hemochromatosis, it is less commonly linked compared to HLA-Dw2.
D. HLA-Dw4: Similar to HLA-Dw2, HLA-Dw4 is less commonly linked with hemochromatosis compared to HLA-Dw2.
**Clinical Pearl:**
HLA (Human Leukocyte Antigen) antigens are proteins found on the surface of immune system cells that play a role in immune response. Certain HLA antigens, such as HLA-Dw2, are associated with an increased risk for hemochromatosis, a genetic disorder affecting iron homeostasis, leading to liver damage and cirrhosis if left untreated. Identifying the patient's HLA type can help guide the diagnosis and inform treatment decisions, as certain HLA types may indicate a higher risk for severe disease progression.
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