ALK-positive anaplastic large cell lymphoma

Case contributed by Yaïr Glick

Presentation

Abdominal and right inguinal pain, nonproductive cough. Painful enlarged right inguinal lymph node biopsied a couple of weeks earlier.

Patient Data

Age: 40 years
Gender: Female

Lymphadenopathy at several mediastinal stations; left supraclavicular up to 13 mm in short axis diameter (SAD); right lower paratracheal up to 14 mm; subcarinal up to 15 mm. Supradiaphragmatic-internal mammary lymph nodes up to 9 mm and in the posterior inferior mediastinum. Mild bilateral hilar, interlobar, and lobar adenopathy.
Hepatic hilar adenopathy up tp 23 mm and bi-iliac - up to 21 mm at the right external iliac chain and 15 at the left one. Retroperitoneal adenopathy, including a conglomerate of para-aortic lymph nodes.
Bilateral enlarged, vividly enhancing inguinal lymph nodes, partly creating conglomerates.

Extensive pulmonary reticulonodular thickening, with bibasal predominance. Minute pleural effusions, right greater than left.

Mild splenomegaly.

Small-to-moderate amount of thick intraperitoneal fluid in pelvis.

Retroverted uterus.

Case Discussion

A young, healthy woman arrived at the ER complaining of unrelenting right inguinal pain, abdominal fullness and pain, and nonproductive coughing. Bilateral enlarged cervical and inguinal lymph nodes were palpated. Since she did not report any B symptom, and the lymph nodes seemed to wax and wane, the possibility of granulomatous disease (i.e. sarcoidosis and tuberculosis) was entertained.

A couple of weeks earlier, the patient had complained of a painful right inguinal lump. An enlarged, mobile right inguinal lymph node was lanced, but the material received was insufficient for investigation. An incision was made above it and, seeing as there were several enlarged inguinal lymph nodes, one was removed and sent to pathology.

A CT chest-abdomen-pelvis was done, revealing chest, abdominal, pelvic, and bilateral inguinal lymphadenopathy, as well as reticulonodular interstitial pulmonary infiltrates.

She tested negative for tuberculosis, HIV, and every other pathogen on the extensive infectious disease panel, except for PCR for parainfluenza virus, which came back positive and explained the interstitial pulmonary disease. Chemistry panel remarkable mainly for elevated hepatocellular enzymes, which then normalized spontaneously.

A few days later, during hospitalization, the histopathology report was ready:

Anaplastic large cell lymphoma, ALK-positive.
The lymph node contains a sheet of large atypical cells. Necrosis is present. The large atypical cells are positive for CD30, CD45 (LCA), CD99, and ALK and are negative for CD2, CD3, CD5, CD7, CD8, CD20, CD43, CD56, CD68, TIA1, Cytokeratin and S100. Ki67 is increased.

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