A case of multiple myeloma with plasma cell cannibalism and cytophagocytosis
Notice bibliographique
Résumé
A 60-year-old woman presented to our hospital complaining of fatigue, confusion, and left sided hip pain. At presentation, bicytopenia was noted with anemia and thrombocytopenia (77 g/L and 33 × 109/L, respectively). She was also hypercalcemic with a new acute kidney injury (2.85 mmol/L and 370 micromol/L, respectively). Serum electrophoresis showed an IgG lambda monoclonal protein in the early gamma region (3.9 g/L) with background immunosuppression. The ratio of the lambda to kappa light chains was markedly elevated (8087 mg/L versus 182 mg/L). This was in conjunction with multiple new lytic lesions evident on her left iliac bone, right 8th rib, and L3 spinous process (first row, panel [A], abdominal computed tomography; a 4.7 cm destructive lesion in the iliac bone is marked). An urgent bone marrow biopsy was requested and performed. The aspirate (stained by May-Grünwald- Giemsa x 50 objective panels B-E) showed a large plasma cell infiltrate (85%). Plasma cells were moderately sized and showed abundance of blue/grey occasionally fraying cytoplasms. These cells also possessed round nuclear contours with relatively fine chromatins and occasional large nucleoli. Interestingly plasma cell cytophagocytosis was evident. Specifically cellular ingestion of platelets, orthochromic normoblasts (top row middle panel [B], black, and white arrows), erythrocytes (top row, third panel [C], white arrow; also note presence of Russel/Dutcher bodies black arrows, respectively), neutrophils (middle row, first panel [D]), and frank plasma cells (middle row and panel [E], grey arrow) were identified. A trephine biopsy showed a hypercellular bone marrow with a large plasma cell infiltrate. In situ cytophagocytosis was identified (Hematoxylin & Eosin stain, middle row third panel [F], white arrow). Cluster of differentiation (CD) 138, kappa, lambda stains showed a near subtotal replacement of bone marrow with lambda restricted plasma cells (bottom row panels (G-I) respectively, x10 objective). Myeloma fluorescence in situ hybridization (FISH) showed the presence of IGH/MAF fusion (t(14;16)(q32.3;q23)), four copies of FGFR3, three copies of CCND1, and relative loss of TP53. Cytogenetic studies showed a complex karyotype: 79–81,XX,add(1)(q21)x2,+2,+2,+3,+3,+5,+5,der(6)t(6;7)(q21;q11.2),+7,+9,+10,+10, del(11)(q21q23),+add(11)(p15)x2,del(12)(q13)x2,add(14)(q32)x2,+add(15)(p11.2)x2,+add(16)(p11.1),+17,+del(17)(p11.2),+18,+18,+19,+19,+20,+20,+21,+21,+der(22)t(4;22)(q12;p11.2)x2,+der(?)t(1;?)(q21;?)x2,+mar1,+mar2,+mar3,+mar4[cp7]/83,XX,add(1)(q21)x2,+3,+3,+5,+5,add(6)(q25)x2,+7,+7,add(8)(q13)x2,add(8)(q22)x1-2,+9,+9,+10,+10,+add(11)(p15)x2,+add(12)(q15)x2,add(14)(q32)x2,+15,+15,+add(16)(p11.1)x2,+17,+del(17)(p11.2),+18,+18,+19,+19,+19+20,+20,+21,+21,+der(22)t(4;22)(q12;p11.2)x2,+der(?)t(1;?)(q21;?)x2,+mar1,+mar2,+mar3,+mar4[cp3]nuc ish(TP53 × 2,D17Z1 × 3)[42/100]/(TP53 × 2,D17Z1 × 4)(52/100),(FGFR3 × 4,IGHx2)[77/100],(CCND1 × 3,IGHx2)[85/100],(IGHx2,MAFx4)(IGH con MAFx2)[77/100]. As her initial blood film showed mild schistocytosis, and with renal injury and confusion, diagnosis of thrombotic thrombocytopenic purpura was considered. However, her haptoglobin and ADAMTS-13 levels were normal, and her red cell fragmentation was attributed to hemophagocytosis. Following the diagnosis of R-ISS stage III lambda light chain multiple myeloma, the patient was treated with three cycles of cyclophosphamide, bortezomib, and dexamethasone therapy. As well, a melphalan-conditioned autologous stem cell transplant is scheduled. Extremely rare, myeloma-related hemophagocytosis has not been associated with a specific immunoglobulin or light chain subtype. Although the presence of identifiable cytogenetic abnormalities is consistent with a more proliferative potential, it is unclear whether an association with phagocytosis exists. Speculation about the utility of aberrant expression of CD15 and CD56 in plasma cells phagocytosis is present in the literature. While the exact mechanism of phagocytosis is not clear, expansion of B cell clones with innate phagocytic potential has been considered. Notwithstanding this proposition needs to be confirmed.
Récupéré en direct depuis OpenAlex et désinversé. Les résumés ne sont pas conservés dans cette base de données : les index inversés représentent 8,6 Go des 9,3 Go de texte de la base, et le serveur dispose de 13 Go libres.
Comment cette classification a été obtenuedéplier
Prédiction distillée sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Apprise à partir de 10 348 étiquettes directes de Codex et de 10 348 étiquettes directes de Gemma. Le mode candidate est l'union des têtes enseignantes seuillées; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont ni des étiquettes humaines ni des étiquettes directes de modèles de pointe.
Scores Codex et Gemma par catégorie
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,000 | 0,000 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,000 | 0,000 |
| Bibliométrie | 0,000 | 0,000 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,000 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule tête enseignante, pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».