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Per rule PH11, "Code the primary site to the site of origin (lymph node region(s), tissue, or organ) and code the histology diffuse large B-cell lymphoma (DLBCL) (9680/3) when DLBCL and any other non-Hodgkin lymphoma are present in the same lymph node(s), lymph node region(s), organ(s), tissue(s) or bone marrow."

Should the focal diffuse large B cell lymphoma be ignored in this case and rule PH11 not be applied? To apply rule PH11, does the follicular lymphoma have to be NOS [9690/3] or does PH11 include all grades of follicular lymphoma [9695/3, 9691/3, 9698/3]?

2021 Bone marrow biopsy showed erythroid hyperplasia, increased histiocytes with hemophagocytosis and Factor XIIIa positive histocytic cells. Moderate cytoplasmic staining for ALK 1, consistent with bone marrow involvement of ALK-positive histiocytosis. A subsequent kidney lesion biopsy was also found to have ALK-positive histiocytosis. The patient was then treated with clofarabine.

Patient is 3 years old.

07/2020-Chart indicates patient presented in June with fevers and refusing to walk with pancytopenia, bone marrow biopsy showed no leukemia buthistiocytes. Impression: ALK positive histiocytosis involving BM and kidney.

10/2020 Bone marrow final diagnosis states right and left bone marrow aspirates and biopsies: No morphologic or immunohistochemical evidence of involvement by the patient's previously diagnosed ALK+ histiocytosis (see Comments) - Multiple histiocytic collections with prominent hemosiderin; favor reactive - background normocellular bone marrow with maturing trilineage hematopoiesis.

The patient's prior bone marrow samples are reviewed (9/2020 and 7/2020). Similar to the September bone marrow sample, the current marrow shows numerous histiocyte collections with abundant associated hemosiderin deposition. These histiocytes have a stellate/dendritic appearance and lack the atypical features noted in the patient's marrow at diagnosis, favoring a reactive process. This impression is further supported by the lack of immunoreactivity for either Factor XIIIa or ALK1 among these cells. There is no convincing morphologic or immunohistochemical evidence of marrow involvement by the patient's previously diagnosed ALK+ histiocytosis within the sampled material. Of note, the marrow otherwise appears normocellular for the patient's age, indicative of ongoing marrow recovery post therapy.

It is not clear whether this would be equivalent to Langerhans cell histiocytosis, disseminated (9751/3) as there is not a statement of Langerhans cell or whether this is just histiocytosis, NOS and not reportable.

Patient complained of pain in the right side and back right upper flank area. CT shows an anterior mediastinal mass and abnormal appearance of skeleton. CXR: Age indeterminate T8 compression fracture. CT chest: abnormal appearance of skeleton. Correlate clinically for myeloma or mets. Acute T5 or T8 compression fractures. Anterior mediastinal mass which may represent thymoma, lymph nodes or metastases. 03/22/12 Metastatic Series: Nonspecific hypodensities in pelvis, left hip and right acromion. Possibility of myeloma can't be totally excluded. Bone marrow right post iliac crest core biopsy, clot section and aspirate: plasma cell myeloma.

Should the primary site be coded to the bone marrow because the diagnosis of plasma cell myeloma was supported by radiologic evidence of multiple lytic lesions? The bone marrow biopsy confirmed the radiology reports.

Patient underwent excision of an oropharyngeal soft tissue mass revealing plasma cell neoplasm with extensive amyloid deposition. During work-up, bone marrow biopsy also revealed involvement by plasma cell neoplasm, with 5-10% of marrow cellularity. No amyloid seen in bone marrow. Patient was referred for radiation of the oropharyngeal mass. Per medical oncology qualifying best for the diagnosis of solitary extramedullary plasmacytoma with minimal marrow involvement. Decision made for observation by medical oncology in view of “minimal” bone marrow involvement. Question: Is rule M11 correct, and I abstract this case as a plasma cell myeloma, 9732/3, C421?

Amyloidosis is not reportable per the Commission on Cancer guidelines. However, the medical oncologist at this facility states that it is a malignancy. The oncologist presented a case at Cancer Conference and indicated the patient has Stage III disease. Should this case be accessioned?

Polycythemia, NOS is not reportable. However, there is a statement in the Heme Manual Glossary for JAK2 that states, "When JAK2 is positive, the MPN is definitely reportable." Does a positive JAK 2 always mean there is a reportable myeloproliferative disorder or must there also be an associated statement of a reportable neoplasm (e.g., myeloproliferative disorder, polycythemia vera, or essential thrombocythemia)?