CASE IS OPEN:
57 yrs old lady with previous splenectomy due to unclassified histiocytic tumor in 2014(linked case No.561404). Persisting anemia and thromocytopenia (after splenectomy)(18). Intravenous Ig not effective, eltrombopag and after- romiplastin. SPLEEN TUMOR (2014) IH: CD4+; CD68+; Lysozyme+; CD1a/S100(-); CD23/CD21(-). Ki67 prolif. activity low <5% (mainly higher on erythroids, scattered histiocytic nuclei). PET scan: LIVER: right lobe: subtotal metabolically active zone SUVmax 11.69. Hilus: several nodes SUVmax 7.82. All bone marrow: infiltration SUVmax 8.77. CT scan: Kepenų S5/6/8 heterogenous tumor with capsular retraction enlarged up to 81 x 57 mm (previously 59 x 46 mm). hilar node up to 26 x 15 mm (previously ~26 x 13 mm.). Splenectomy place: ~32x29 mm ir 18x16 mm "splenulae" vs MTS. Bone structure effaced with irregular focci. LIVER BIOPSY: Confluent zones of spindled and pale epithelioid histiocytes with reniform, ovoid, pleomorphic nuclei. Not typed fully: CD68+; Ki67 low ~1%; Asm Actin/Desmin/H-caldesmon, CD34/CD31/S100/ALK/STAT6(-). BONE MARROW: a) Bone marrow panhyperplasia (after age) with architectural damage, erythroid hiperplasia/megaloblastic dyserythropoiesis, minimal Fe deposition, TdT+ hematogone excess: reactive changes (B12/Folic deficiency? other?). b) Atypical histiocytic infiltration, ~5% BM. IH: CD68/CD163/CD4+; CyclinD1+; p53+; S100/CD1a/CD11c(-). MOLECS (retrospectively from spleen): BRAF mutations not detected. BM: SF3B1 not detected in the region R590-G740. NGS (solid targeted panel): no significant abberrations detected. IgH K L and TCR clonality not detected (in 2014). DIAGNOSTIC QUESTION: Definite NOSOLOGIC position of disseminated (indolent/low Ki67) histiocytosis (non Langerhans)? Annotations
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Last modified: 2019-04-05 15:49:02