RESUMO
Chronic myelomonocytic leukemia (CMML) is a rare disease of clonal hematopoietic stem cells with an inherent risk of leukemic transformation, seen in an elderly male. Case Presentation: Herein, the authors report a case of CMML in a 72-year-old male who presented with fever and abdominal pain for 2 days with a history of easy fatigability. Examination revealed pallor and palpable supraclavicular nodes. Investigations showed leukocytosis with a monocyte count of 22% of white blood cell count, 17% blast cells in bone marrow aspiration, increased blast/promonocytes, and positive markers in immunophenotyping. The patient is planned for injection of azacitidine, 7 days cycle for a total of six cycles. Clinical Discussion: CMML is classified as overlapping myelodysplastic/myeloproliferative neoplasms. It can be diagnosed based on a peripheral blood smear, bone marrow aspiration and biopsy, chromosomal analysis, and genetic tests. The commonly used treatment options are hypomethylating agents like azacitidine and decitabine, allogeneic hematopoietic stem cell transplant, and cytoreductive agents like hydroxyurea. Conclusion: Despite various treatment options, the treatment is still unsatisfactory, demanding standard management strategies.
RESUMO
Although risk factors such as cholelithiasis and vaso-occlusive crises exist in sickle cell disease, acute pancreatitis and its recurrence are considered rare complications manifesting as acute abdomen. Case Presentation: A 33-year-old female with sickle cell disease and established cholelithiasis presented to the center with acute abdomen. After examination, investigation, and contrast enhanced computed tomography, acute pancreatitis was diagnosed. Conservative management was done and cholecystectomy was planned but delayed due to low hemoglobin. In the interval she presented again with similar features and diagnosed with of recurrence. After conservative management and after optimization of patient's hemodynamic status, laparoscopic cholecystectomy was finally performed. Postoperative period and follow-up visit after 2 months were uneventful. Clinical Discussion: As the features of pancreatitis and vaso-occlusive crisis are similar, the former should be considered as a differential diagnosis of acute abdomen in sickle cell patients. Laparoscopic cholecystectomy is the treatment of choice for symptomatic cholelithiasis in these patients. However, some issues related to management such as preoperative transfusion and prophylactic cholecystectomy are still debated. This uncertainty caused delay in surgery which may have contributed to the recurrence of pancreatitis in our patient. As the risk of recurrence is possible after the first attack, standard guidelines are required for the definite management of the cause. Conclusion: This case report adds to the limited literature on recurrent acute pancreatitis in sickle cell patients and points out the need for studies on developing management guidelines in such patients and need for prophylactic treatment.
