Patho-anatomical features of so-called Ph1? chronic myeloid leukemia

Abstract

Chronic myeloid leukemia without the Philadelphia chromosome (Ph1-CML) is described and distinguished from chronic myeloid leukemia with the Philadelphia chromosome (Ph1+CML) on the basis of clinical and autopsy findings of four cases. Ph1-CML showed clinical, hematological, and patho-anatomical features which could be regarded as typical. Patho-anatomically Ph1-CML differed from Ph1+CML in the variable maturation of the leukemic proliferation in the bone marrow and extramedullary infiltrates. Up to the terminal phase Ph1-CML can be of an extremely mature cell type. However, it can also show myeloblastic transformation after an initially mature cell stage. Ph1-CML infiltrates are found in tissues and organs which Ph1+CML usually does not infiltrate or only to a low degree until a blastic crisis. On the basis of its course and clinical and patho-anatomical features Ph1-CML looks like an atypical chronic myeloid leukemia. However, it is better called an acute myeloid leukemia of the mature cell type.

Myeloproliferative Syndromes and Thrombocythemia

“…In part of patients with CML, the liver is infiltrated by the neoplastic cell populations (Hopps and Rucks 1948;Schwarze et al 1975;Walz-Mattm€ uller et al 1998). In addition, patients with CML may show "normal-looking" extramedullary hematopoiesis (Kajigaya et al 1987).…”