Case 902 -57y.o.

man -3mo history of left upper quadrant pain with sensation of fullness (splenomegaly) -9kg weight loss (cancer?) -easy bruisability (thrombocytopenia) -several episodes of sharp left upper quadrant pain aggravated by deep breathing -no lymphadenopathy or hepatomegaly -spleen palpable and tender DDx -Chronic myeloid leukemia: Ages 25-60, median age 55. 15% of leukemia in adults. Enlarged spleen causing pain, low-grade fever, anemia, thrombocytopenia with easy bruising. Risk factors: ionizing radiation and benzene. Clinically see hepatosplenomegaly and generalized painless lymphadenopathy. -myelofibrosis: Occurs in patients >50y.o. most common cause of splenomegaly in this group. Usually have massive splenomegaly with portal hypertension -Hairy cell leukemia: common in middle aged men. Splenomegaly without lymphadenopathy (ONLY leukemia without lymphadenopathy), hepatomegaly in 20% of cases. Pancytopenia -Myelodysplastic syndrome - usually occurs in men between 50-80y.o. Anemia, neutropenia, thrombocytopenia. Splenomegaly (rarely hepatomegaly). Frequently progress to acute myeloblastic anemia -Acute myeloid leukemia common leukemia in persons 40-60 (>60% of cases). CML is 40% of cases CLL most common in individuals >60y.o. Most common overall leukemia. Generalized lymphadenopathy. More common in males Hodgkins lymphoma/Follicular lymphoma dont have any large lymph nodes. Common malignancy in young adults Acute lymphoblastic typically children and young adults Normochromic, normocytic anemia, hemoglobin and hematocrit low Platelets ok WBC extremely elevated Basophilia and Eosinophillia No tear drop RBC, platelets not abnormal (so not myelofibrosis or myelodysplastic) Blasts typically less than 2%
presence of a greater percent of myelocytes than the more mature metamyelocytes ("leukemic hiatus" or "myelocyte bulge") is one of the classic findings in CML

Myelocytes, neutrophils, band cells. Bilobed neutrophil (butterfly) Not CLL because cells are not small or monomorphic Lean toward CML because : Basophilia invariably present, eosinophilia common. Left shift and leukocytosis. 50% asymptomatic. Lethargy fatigue, weight loss. Splenomegaly (50%) Hypercellular bone marrow increased numbers of maturing myeloid cells.

There is usually a widened area of immature neutrophils in the paratrabecular cuff and mature neutrophils are found in the intertrabecular areas. Erythroid islands are reduced in number and size. Small megakaryocytes with hypolobulated nucle Not acute because no block in differentiation the following features are more commonly found in a leukemoid reaction and help to distinguish it from CML: toxic granulation in the neutrophils, a high LAP score, lack of a "myelocyte bulge", and most importantly, the presence of an obvious cause for the neutrophilia.

allopurinol to prevent urate nephropathy from lysis interferon alfa with or without cytarabine for accelerated phase for chronic phase

drugs of choice imatinib interferon alfa with or without cytarabine allogeneic peripheral stem-cell or bone-marrow transplant alternative - hydroxyurea

For accelerated phase

drugs of choice imatinib cytarabine and daunorubicin or idarubicin allogeneic peripheral stem-cell or bone-marrow transplant alternatives hydroxyurea interferon alfa

Varying definitions have been applied to the accelerated phase (table 1) [1]. The most frequently used criteria, the WHO definition, defines accelerated phase as patients with CML who show one or more of the following features: 10 to 19 percent blasts in the peripheral blood or bone marrow Peripheral blood basophils 20 percent Platelets <100,000/microL, unrelated to therapy Platelets >1,000,000/microL, unresponsive to therapy Progressive splenomegaly and increasing white cell count, unresponsive to therapy Cytogenetic evolution (defined as the development of chromosomal abnormalities in addition to the Philadelphia chromosome)