Chediak-Higashi syndrome (CHS) is a rare autosomal recessive disease, characterized by partial oculocutaneous albinism, recurrent pyogenic infections (skin, mucosa and respiratory system), and neurologic deficit. The hallmark of this syndrome is the presence of abnormal intracytoplasmic giant granules in all granule containing cells including leukocytes in blood and bone marrow. A majority (85 %) of patients with CHS develop an accelerated phase consisting of a lymphoproliferative syndrome with hemophagocytosis and infiltration of most tissues. This phase is characterized by fever, jaundice, hepatosplenomegaly, lymphadenopathy, pancytopenia and neurological abnormalities. In this paper, we report a case of CHS presented as accelerated phase in a 9-month-old girl child.

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Background: Spontaneous remission (SR) has been reported in different hematological malignancies. It has been observed in adult T-cell lymphoma, chronic lymphocytic leukemia (CLL) and myelodysplastic syndrome (MDS). It is generally associated with recovery from an infectious or immunological process, and more recently possibly with clonal hematopoiesis. Case: We reviewed the literature and reported a new case of a 40 year-old man with a morphologic and cytogenetic diagnosis of early T-cell precursor acute lymphoblastic leukemia (ALL) associated with an appendicular abscess. During his hospitalization and surgical management of his appendicitis, we noted SR of the rate of blast cells until cytological and cytogenetic remission of his ALL but unfortunately it did not last too long, moreover our patient relapsed after nine months, received intensive chemotherapy, underwent a placental blood allograft but relapsed again and died. Conclusions: In contrast to SR in other types of cancer, all documented cases of SR in ALL were only transient, so is there a need for early cytotoxic therapy in SR in ALL to delay relapse?