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Cold agglutinin disease in fibrolamellar hepatocellular carcinoma: a rare association with a rare cancer variant.

Al-Matham K, Alabed I, Zaidi SZ, Qushmaq KA - Ann Saudi Med (2011 Mar-Apr)

Bottom Line: Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia.Although it can occur secondary to lymphoproliferative disorders and autoimmune or infectious diseases, CAD is rarely reported as secondary to solid tumors.In summary, although it is an extremely rare association and less common than lymphoproliferative disorders, CAD can be associated with solid tumors.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Main Hospital, King Fahad Medical City, Riyadh, Saudi Arabia. khalidosis@hotmail.com

ABSTRACT
Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia. Although it can occur secondary to lymphoproliferative disorders and autoimmune or infectious diseases, CAD is rarely reported as secondary to solid tumors. We report a case of a woman aged 18 years diagnosed with a well-differentiated hepatocellular carcinoma of the fibrolamellar subtype, who was shown to have CAD also. Her general condition, including CAD, improved after targeted therapy with sorafenib for the hepatocellular carcinoma and only conservative measures for the CAD that consisted of avoidance of cold. In summary, although it is an extremely rare association and less common than lymphoproliferative disorders, CAD can be associated with solid tumors.

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Liver biopsy showing hepatocellular carcinoma: fibrolamellar subtype (Hematoxylin and eosin stain ×200).
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Figure 4: Liver biopsy showing hepatocellular carcinoma: fibrolamellar subtype (Hematoxylin and eosin stain ×200).

Mentions: A woman aged 18 years presented with right upper quadrant abdominal pain, vomiting, yellowish discoloration of the sclera, dark urine, and weight loss and poor appetite for the previous 3 months. Examination showed pallor, one small (1.0×1.0 cm) left supraclavicular lymph node, right upper quadrant tenderness, and hepatomegaly (liver span, 16 cm). Laboratory investigations showed elevated red cell indices (artifactual); white blood cells, 11.6×109/L (normal reference range, 4-11×109/L); platelets, 526×109/L (reference range, 140-450×109/L); erythrocyte sedimentation rate, 150 mm/h (reference range, 0-20); C-reactive protein, 12 mg/L (0-5.99) reticulocytes, 5.4% (0.5-1.5% of RBCs); alanine aminotransferase, 102 U/L; aspartate aminotransferase, 122 U/L; alkaline phosphatase, 187 U/L; total bilirubin, 67.2 mmol/L; direct bilirubin, 7.3 mmol/L; ferritin, 514.3 (g/L; vitamin B12, 1034 pmol/L; and lactate dehydrogenase, 292 U/L; with decreased levels of albumin (28 g/L) and hemoglobin 7.6 g/dL). Blood film examination revealed strong agglutination of RBCs, which is characteristic of CAD. Serum IgA was elevated, while IgG and IgM concentrations were within normal ranges. Negative results were obtained for hepatitis B and C, HIV and EBV (by polymerase chain reaction). Mycoplasma pneumonia serology was negative. Alpha-fetoprotein levels were normal. Results of the direct Coombs test and cold agglutinin tests were strongly positive (specific titer not obtained). Chest X-ray was normal. CT abdomen showed a 12×16-cm liver mass with multiple enlarged lymph nodes (Figure 1). Liver biopsy showed a well-differentiated hepatocellular carcinoma of the fibrolamellar subtype (Figures 2 a, b, c). Bone marrow aspiration and biopsy results were normal except for erythroid hyperplasia. A lymph node biopsy from the para-aortic lymph node showed total replacement and destruction of architecture by adjacent tumor tissue (local metastasis).


Cold agglutinin disease in fibrolamellar hepatocellular carcinoma: a rare association with a rare cancer variant.

Al-Matham K, Alabed I, Zaidi SZ, Qushmaq KA - Ann Saudi Med (2011 Mar-Apr)

Liver biopsy showing hepatocellular carcinoma: fibrolamellar subtype (Hematoxylin and eosin stain ×200).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3102484&req=5

Figure 4: Liver biopsy showing hepatocellular carcinoma: fibrolamellar subtype (Hematoxylin and eosin stain ×200).
Mentions: A woman aged 18 years presented with right upper quadrant abdominal pain, vomiting, yellowish discoloration of the sclera, dark urine, and weight loss and poor appetite for the previous 3 months. Examination showed pallor, one small (1.0×1.0 cm) left supraclavicular lymph node, right upper quadrant tenderness, and hepatomegaly (liver span, 16 cm). Laboratory investigations showed elevated red cell indices (artifactual); white blood cells, 11.6×109/L (normal reference range, 4-11×109/L); platelets, 526×109/L (reference range, 140-450×109/L); erythrocyte sedimentation rate, 150 mm/h (reference range, 0-20); C-reactive protein, 12 mg/L (0-5.99) reticulocytes, 5.4% (0.5-1.5% of RBCs); alanine aminotransferase, 102 U/L; aspartate aminotransferase, 122 U/L; alkaline phosphatase, 187 U/L; total bilirubin, 67.2 mmol/L; direct bilirubin, 7.3 mmol/L; ferritin, 514.3 (g/L; vitamin B12, 1034 pmol/L; and lactate dehydrogenase, 292 U/L; with decreased levels of albumin (28 g/L) and hemoglobin 7.6 g/dL). Blood film examination revealed strong agglutination of RBCs, which is characteristic of CAD. Serum IgA was elevated, while IgG and IgM concentrations were within normal ranges. Negative results were obtained for hepatitis B and C, HIV and EBV (by polymerase chain reaction). Mycoplasma pneumonia serology was negative. Alpha-fetoprotein levels were normal. Results of the direct Coombs test and cold agglutinin tests were strongly positive (specific titer not obtained). Chest X-ray was normal. CT abdomen showed a 12×16-cm liver mass with multiple enlarged lymph nodes (Figure 1). Liver biopsy showed a well-differentiated hepatocellular carcinoma of the fibrolamellar subtype (Figures 2 a, b, c). Bone marrow aspiration and biopsy results were normal except for erythroid hyperplasia. A lymph node biopsy from the para-aortic lymph node showed total replacement and destruction of architecture by adjacent tumor tissue (local metastasis).

Bottom Line: Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia.Although it can occur secondary to lymphoproliferative disorders and autoimmune or infectious diseases, CAD is rarely reported as secondary to solid tumors.In summary, although it is an extremely rare association and less common than lymphoproliferative disorders, CAD can be associated with solid tumors.

View Article: PubMed Central - PubMed

Affiliation: Department of Internal Medicine, Main Hospital, King Fahad Medical City, Riyadh, Saudi Arabia. khalidosis@hotmail.com

ABSTRACT
Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia. Although it can occur secondary to lymphoproliferative disorders and autoimmune or infectious diseases, CAD is rarely reported as secondary to solid tumors. We report a case of a woman aged 18 years diagnosed with a well-differentiated hepatocellular carcinoma of the fibrolamellar subtype, who was shown to have CAD also. Her general condition, including CAD, improved after targeted therapy with sorafenib for the hepatocellular carcinoma and only conservative measures for the CAD that consisted of avoidance of cold. In summary, although it is an extremely rare association and less common than lymphoproliferative disorders, CAD can be associated with solid tumors.

Show MeSH
Related in: MedlinePlus