DUODENAL WALDENSTROM MACROGLOBINEMIA

Case Provided by: Angela Darko, M.D. and James Carlsten, M.D.

Case history

77 year old female with no prior medical history presented with anemia, early satiety, abdominal distension and elevated sedimentation rate. She had no weight loss, melana or hematochezia. She also had no previous medical history. Her hematology laboratory values showed a WBC of 6.8 with 27% lymphocytes on manual differential. Post-transfusion values showed elevated WBC of 12.7 with 46 % lymphocyte. Endoscopy findings included erythema and edema of the duodenal folds

Microscopic findings

The biopsy consisted of fragments of duodenal mucosa with abundant intra-lymphatic deposits of acellular eosinophilic material within the tips of the villi and within the base of the mucosa. These deposits were PAS positive, diastase resistant, and IGM immunohistochemical stain positive. A Congo red stain was negative for amyloid

Diagnosis

Duodenal Waldenstrom’s macroglobulinemia

Discussion

Waldenstrom macroglobinemia discovered by Jan Waldenstrom in 1944 is a rare indolent lymphoplasmacytic lymphoma. There are 1500 new cases in the United States annually and the median age of diagnosis is 63 years

The clinical manifestation of Waldenstrom is produced by the high monoclonal IgM gammopathy that can cause visual and neurologic symptoms. It can also present as small lymphocytic intertrabecular infiltration of the bone marrow and other tissues.

In the gastrointestinal tract the monoclonal IgM deposits in the mucosa and lymphatic’s leading to malabsorption, diarrhea and protein-loosing enteropathy if it is diffuse. The symptoms can be mild to severe and may be the only presenting symptoms that ultimately leads to a diagnosis systemic Waldenstrom Macroglobinemia.

Endoscopically the lesions may be seen as small white-grey granular mucosal nodules. The microcopic features include mild variable villous blunting, mildly increased lymphoplasmacytic infiltrate, and dilated villous and mucosal lymphatic’s filled with densely eosinophic acellular amorphous deposits. Occasional atypical lymphocytes and foamy macrophages may be seen in the lamina propria. The deposits are negative for Congo red, but are positive for diastase resistant PAS. They are strongly positive for IgM immunohistochemical stain and can show immunoglobulin light chain restriction on lambda or kappa in-situ hybridization stains.

The differential diagnosis includes amyloidosis, lymphagiectasia, Whipple’s disease, Mycobacterium avium intercellulare, and other conditions that cause increased macrophages. Amyloidosis will have Congo-red positive eosinophilic deposition around vessels. Lympangiectasisa shows dilated lymphatics without the eosinophilic deposits. Mycobacterium avium intercellulare shows foamy AFB-fite positive organisms in macrophages distending the lamina propria. Whipple’s disease shows Pas positive foamy macrophages filled with actinomycete, Tropheryma whippelii. This can be confirmed with PCR for amplification of the 16S rRNA sequence of the bacteria.

A peripheral blood smear evaluation and flow cytometry were subsequently performed and the patient was diagnosed with a non-Hodgkin’s B-cell lymphoma.

References

Surgical pathology of the GI tract, liver, biliary tract, and pancreas / [edited by] Robert D. Odze, John R. Goldblum.—2nd ed.

Bedine MS, Yardley JH, Elliott HL, et al: Intestinal involvement in Waldenström's macroglobulinemia. Gastroenterology 1973; 65:308-315

Pruzanski W, Warren RE, Goldie JH, et al: Malabsorption syndrome with infiltration of the intestinal wall by extracellular monoclonal macroglobulin. Am J Med 1973; 54:811-818.

Pratz KW, Dingli D, Smyrk TC, et al: Intestinal Lymphangiectasia With Protein-Losing Enteropathy in Waldenstrom Macroglobulinemia. Medicine:July 2007 - Volume 86 - Issue 4 - pp 210-214

 

DUODENAL WALDENSTROM MACROGLOBINEMIA
DUODENAL WALDENSTROM MACROGLOBINEMIA
DUODENAL WALDENSTROM MACROGLOBINEMIA
DUODENAL WALDENSTROM MACROGLOBINEMIA
DUODENAL WALDENSTROM MACROGLOBINEMIA
DUODENAL WALDENSTROM MACROGLOBINEMIA
DUODENAL WALDENSTROM MACROGLOBINEMIA
DUODENAL WALDENSTROM MACROGLOBINEMIA