Journal of Hematology, ISSN 1927-1212 print, 1927-1220 online, Open Access
Article copyright, the authors; Journal compilation copyright, J Hematol and Elmer Press Inc
Journal website https://www.thejh.org

Case Report

Volume 13, Number 5, October 2024, pages 216-223

Transformation of Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma to Histiocytic/Dendritic Cell Sarcoma

Figures

Figure 1.
Figure 1. Photomicrograph of the ultrasound-guided core biopsy of an enlarged lymph node in the posterior triangle of the right neck shows lymphoid tissue with many paler areas. (H&E stain, original magnification, × 100). H&E: hematoxylin and eosin.
Figure 2.
Figure 2. Photomicrographs of the ultrasound-guided core biopsy of an enlarged lymph node in the posterior triangle of the right neck. The paler areas contain sheets of atypical histiocytic/dendritic cells with irregular nuclear contours and prominent/distinct nucleoli. There are occasional mitotic figures, including atypical ones (a, short arrow). Small mature lymphocytes and scattered eosinophils (b, long arrows) are admixed (H&E stain, original magnification, (a) × 200, (b) × 400). H&E: hematoxylin and eosin.
Figure 3.
Figure 3. Immunohistochemical (IHC) characterization of the tumor. The histiocytic/dendritic cells are positive for CD4 (a), CD45 (not shown), CD68 (granular and Golgi pattern (b), CD163 (small subset, not shown), BCL6 (c), cyclin D1 (d), lysozyme (e), and S-100 (f), weakly positive for OCT-2 (g) and BCL2 (subset, not shown), and were negative for CD1a (not shown), CD3 (not shown), CD5 (not shown), CD10 (not shown), CD20 (not shown), CD21 (not shown), CD23 (not shown), CD30 (not shown), CD79a (not shown), ALK1 (not shown), BOB.1 (h), MUM1 (not shown), PAX-5 (not shown), and cytokeratin AE1/AE3 (not shown). (i) Ki67 IHC stain shows a high proliferation rate (immunoperoxidase staining; original magnification, × 200).
Figure 4.
Figure 4. A small focus of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) by immunohistochemistry. The CLL/SLL cells are negative for CD3 (a), but express CD5 (b), CD20 (c), and CD23 (d). The background T-lymphocytes are positive for both CD3 and CD5 (immunoperoxidase staining; original magnification, × 200). CLL/SLL: chronic lymphocytic leukemia/small lymphocytic lymphoma.

Tables

Table 1. Blood Test Results at Harbor-UCLA Medical Center at Presentation of Progressive Lymphadenopathy
 
TestResultNormal range
INR: international normalized ratio.
Sodium139 mmol/L136 - 144 mmol/L
Potassium4.3 mmol/L3.6 - 5.1 mmol/L
Chloride107 mmol/L101 - 111 mmol/L
CO227 mmol/L22 - 32 mmol/L
Blood urea nitrogen (BUN)13 mg/dL8 - 20 mg/dL
Creatinine0.91 mg/dL0.64 - 1.27 mg/dL
Calcium9.3 mg/dL8.9 - 10.3 mg/dL
Phosphorous4.0 mg/dL2.4 - 4.7 mg/dL
Magnesium2.1 mg/dL1.8 - 2.5 mg/dL
Total protein6.6 g/dL6.4 - 8.3 g/dL
Albumin3.7 g/dL3.5 - 4.8 g/dL
Immunoglobulin A285 mg/dL40 - 350 mg/dL
Immunoglobulin G1,180 mg/dL650 - 1,600 mg/dL
Immunoglobulin M54 mg/dL50 - 300 mg/dL
Alkaline phosphatase65 U/L38 - 126 U/L
Aspartate transaminase (AST)14 U/L15 - 41 U/L
Alanine transaminase (ALT)15 U/L10 - 40 U/L
Total bilirubin0.4 mg/dL0.3 - 1.2 mg/dL
Bilirubin, direct< 0.1 mg/dL0.1 - 0.5 mg/dL
Haptoglobin249 mg/dL36 - 195 mg/dL
Glucose101 mg/dL74 - 118 mg/dL
Beta-2 microglobulin1.4 mg/L0.8 - 2.2 mg/L
Prothrombin time (PT)13.4 s11.5 - 14.5 s
INR1.040.86 - 1.15
Partial thromboplastin time (PTT)26.1 s23.4 - 34.8 s
Fibrinogen545 mg/dL215 - 450 mg/dL
Lactate dehydrogenase154 U/L98 - 192 U/L
Uric acid6.7 mg/dL2.6 - 8.0 mg/dL

 

Table 2. Immunologic Characterization of the Histiocytic/Dendritic Cells by Immunohistochemistry
 
CD1a-
CD3-
CD4+
CD5-
CD10-
CD20-
CD21-
CD23-
CD30-
CD45+
CD68+ (granular and Golgi pattern)
CD79a-
CD163+ (small subset)
ALK1-
BCL2+ (subset, weak)
BCL6+
Bob.1-
Cyclin D1+
Cytokeratin AE1/AE3-
Ki67+ (high)
Lysozyme+
MUM1-
OCT-2+ (weak)
PAX-5-
S-100+

 

Table 3. Treatment Regimens and Responses by Line of Therapy
 
Line of therapyTreatment regimenTreatment response
FirstIfosfamide, carboplatin, etoposide (ICE) × 3 cyclesPartial response
SecondRituximab, etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin (R-EPOCH) + zanubrutinib × 2 cycles (at the time of manuscript, one cycle was administered)To be determined
Followed by allogeneic hematopoietic stem cell transplantation (HSCT) (planned)