Avascular Necrosis of Femoral Head as the Initial Manifestation of CML

Abstract

A 12-year-old female is reported who presented with right hip pain for 6 months. With massive splenohepatomegaly and leukocytosis, CML was suspected and confirmed on bone-marrow examination and cytogenetics. Further investigations confirmed avascular necrosis (AVN) of the right femoral head. CML was treated by hydroxyurea, followed by imatinib. AVN was managed conservatively; patient demonstrated progressive improvement, though a mild limp in the gait was persisting at 22 months. AVN as the initial manifestation of CML is a rarity. Leukostasis is considered to be the pathophysiological mechanism. In view of the rarity, a case is reported, along with compilation of previously reported cases.

Leukostasis-Related Fatal Cardiopulmonary Arrest as Initial Chronic Myeloid Leukemia Presentation

“…Rottenstreich et al reported a case of bilateral calf pain and retinal hemorrhages due to leukostasis in a young CML male [22] and Awh et al reported a case with leukostasis related retinopathy in a 41-year-old man with CML and severe hyperleukocytosis successfully treated with dasatinib [23]. Three different groups [6,24,25] presented three cases of femoral necrosis as initial presentation of CML with hyperleukocytosis. Tsai et al reported a sudden hearing loss as the initial manifestation of CML in a child [26], while Woldag et al reported a labyrinth apoplexy in a 45-year-old patient with CML and hyperleukocytosis [27] and Frankel et al reported a case of "acral lividosis" in a similar patient [28].…”

Early presentation of osteonecrosis in acute lymphoblastic leukemia: Two children from the Nordic and Baltic cohort

“…Leukostasis, thrombocytosis, and bone marrow hypercellular infiltration have been proposed to induce early ON development. In combination with changes in bone homeostasis during puberty, these changes caused by leukemia could create a state of ischemia in the bone that could induce ON.…”

“…Earlier progression to puberty in females compared with males alters hormone levels, growth plate maturation, and lipid metabolisms. 5 Leukostasis, thrombocytosis, 16 and bone marrow hypercellular infiltration 10,17 have been proposed to induce early ON development.…”

“…In combination with changes in bone homeostasis during puberty, Most reported patients with early manifestation of ON have had chronic myeloid leukemia. 16,17,[21][22][23] Only one adult male with ALL and early ON has been described; the ON progression was relatively benign. 10 In contrast, one child described here had ongoing joint pain and ON in both knees 2 years after end of ALL therapy, suggesting that ON manifesting early can progress into more severe ON during ALL treatment.…”

“…Most reported patients with early manifestation of ON have had chronic myeloid leukemia . Only one adult male with ALL and early ON has been described; the ON progression was relatively benign .…”