Auer rods are needle- or rod-shaped crystalline structures derived from myeloid cell granules. They are most commonly seen in acute myeloid leukemia (AML), but are also associated with other, high-grade myelodysplastic syndromes and neoplasms.
Auer rods indicate the lineage and neoplastic nature of the ongoing disease. Bundles of unusually long Auer rods are very characteristic (but not totally specific) for AML with t(8;21)(q22;q22) translocation.
CNS involvement in ALL usually takes the form of relapse and occurs in 5-10% of cases. CNS positivity at the time of diagnosis is associated with a worsened prognosis.
CNS involvement presents with nonspecific neurologic complaints such as headache, dizziness, visual field deficits, or as leukemic meningitis. Rarely, parenchymal hemorrhage due to vascular infiltration of leukemic cells can be the presenting picture.
The central nervous system is a sanctuary for ALL, and CNS prophylaxis is an essential part of therapy.
Cranial radiation therapy (CRT) was once standard for all ALL patients but has been largely replaced by intrathecal or systemic chemotherapy in lower-risk patients due to the risk of secondary brain tumors, endocrinopathies and neurocognitive deficits.
Bone marrow biopsy is used to establish the diagnosis of ALL and identify its subtype.
The normal bone marrow is comprised of less than 5% of blasts, while leukemic marrow is hypercellular and almost completely infiltrated with leukemic blasts.
ALL should be suspected if the marrow contains more than 5% blasts, but the diagnosis should not be made on a single smear showing less than 25% blasts.
Over recent years, the intensity of induction therapy has increased.
The original combination of vincristine and a steroid has evolved into a regimen consisting of vincristine, a steroid and an anthracycline such as daunorubicin, or quadruple therapy, which adds an asparaginase product such as pegylated asparaginase.
Complete remission is possible in 85-95% of cases, while the remainder will suffer from persistent disease or death.
Band keratopathy is a chronic degenerative disease that can be associated with many disorders, especially inflammatory conditions such as Stills disease (juvenile rheumatoid arthritis). It is characterized by deposition of gray to white opacities in the superficial layers of the cornea, and develops over months to years.
While the underlying pathophysiology remains unknown at the time of this writing, it appears to represent a calcifying tendency of the comorbid condition.
Hypercalcemia of malignancy is rare in children, but has been reported in rhabdomyosarcoma, hepatoblastoma, Hodgkin's and non-Hodgkin's lymphoma, brain tumors, neuroblastoma, angiosarcoma, ALL and AML.
It is an extremely rare primary presentation of ALL, and thus may prolong the diagnosis. It usually entails a non-specific constellation of nausea, vomiting, abdominal pain, anorexia, constipation and weight loss.
Treatment is with the aggressive administration of IV fluid and in more severe cases, IV pamidronate, furosemide, and dexamethasone. Emergency dialysis may be required in refractory cases.
Metastasis is common in the natural course of many malignancies. It results from the ability of tumor to spread from the site of origin to local or distant anatomical sites.
Since leukocytes are able to access nearly all parts of the body for immune protection, it can be inferred that leukemia has an inherent ability to metastasize.
Metastatic activity affecting the bones and or direct trauma to the bones from blast proliferation, as seen in this case, can damage them and induce osteoclastic activity. This increases resorption of calcium into the blood, leading to hypercalcemia.
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