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Generalized histiocytic lesion of JXG type in a patient treated for acute lymphoblastic leukemia with chromosome 5 trisomy



3  Atlas of Rare Lymphomas

3.2  Generalized histiocytic lesion of JXG type in a patient treated for acute lymphoblastic leukemia with chromosome 5 trisomy

Introduction:

9 years old patient with acute lymphoblastic leukemia (ALL) with chromosome 5 trisomy was treated according to contemporary BFM protocol.

History:

1st CR including flow cytometric MRD negativity was achieved within the first month of the therapy. During the late intensification course of his ALL protocol he suffered from prolonged fever of unknown origin, not responding to standard ATB or antimycotic regimens and lasting even after the marrow recovery.

Clinical signs:

At that time multiple brownish skin lesions and focal hepatic lesions were observed.

Bioptical examination of brown skin lesion of the nose as well as nodular hepatic lesion and bone marrow were performed.

Pictures

Papule of the nose:
Juvenile xanthogranuloma after ALL therapy, CLINIC (73928)

Therapy:

Therapy based on LCH-8-98 (refractery LCH) protocol was started, but shortly after that exhausted patient succumbed to Klebsiella ESBL positive sepsis.

Histology:

Biopsy of bone marrow failed to prove leukemia and MRD was negative as well, but revealed the population of bland histiocytes (20%), identical to cells of the biopsied lesion of nose, and liver nodules.

Conclusion:

The case was closed as generalized juvenile xanthogranuloma in a patient treated for acute lymphoblastic leukemia.

Pictures

Skin lesion:
Multicentric histiocytosis, HE 60x (73895)

Multicentric histiocytosis, HE 60x (73774)



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