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CASE REPORT hematology. Monika Csóka MD, PhD. 16 year old boy no abnormalities in previous anamnesis 2 weeks before viral infection (fever, coughing) 1 day before petechias , bruises, hematomas on skin and oral mucosal membranes he was presented in hematology outpatient dept. ?.
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CASE REPORThematology Monika Csóka MD, PhD
16 year old boy • no abnormalities in previous anamnesis • 2 weeks before viral infection (fever, coughing) • 1 day before petechias, bruises, hematomas on skin and oral mucosal membranes • he was presented in hematology outpatient dept.
? • Trombocytopenia, thrombocytopathia • Vasculopathia • leukemia Clinical data Laboratory values
Clinical data • Lymphnodes • Liver • Spleen • Testes • Leukemic infiltration in skin, gingiva • Neurological signs
Lab. • Blood count • LDH • Peripheral blood smear
lab • LDH 1600 U/l • CRP 34 mg/l • GPT 29 U/l • GGT 71 U/l • Kreatinin 110 umol/l • Uric acid 202 umol/l • Smear: thrombocytopenia, no other abnormalities
ITP ? • Bone marrow aspiration not obligatory • But obligatory if corticosteroid treatment is planned • First line treatment: IVIG or corticosteroid (effectivity, side effects, price)
Next step • 16 years • 70 kg • Planned treatment: corticosteroid • Diagnostics: bone marrow aspiration
Surprise!!! • Bone marrow smear: 70% lymphoblast • CD10+, CD19+, CD20+ • Dg: ALL
ALL • Most common malignancy (33%) • ALL 75% • AML 20% • CML (CLL) 5%
Clinical signs • Fever • Anaemia • Bleeding • Lymphadenopathia • Hepatosplenomegalia • Bone pain
Diagnosis • Blood count, smear, lab • Bone marrow • LP
Lab • Anemia • Thrombocytopenia • WBC ( ) • Blasts in peripheral blood smear • LDH • Uric acid
Survival • EFS 5 years 78,6% • SR 96,1% • MR 77,9% • HR 40,8%
Thrombocytopenia • Thrombocytopenia is defined as a platelet count of < 150,000/ μL • Due to: • Increased destruction • Sequestration • Decreased production
Thrombocytopenia • Systematic approach to evaluating patients: • History (associated illness, drugs, specific symptoms) • Physical exam (anomalies, hepatosplenomegaly, infection, tumor, lymphadenopathy, bleeding) • Careful interpretation of the complete blood count and examination of peripheral blood smear • If diagnosis not made or corticosteroid is planned consider bone marrow examination
Case 2 • 14 year old female presented with complaints of: • Easy bruising x 1 month • Heavy menses • 1-2 episodes of fever in the past month • 2 days of cervical lymphadenopathy (resolved) • Occasional night sweats • No weight loss
Case 2 • Initial laboratory findings: • WBC: 3.15 x 1000/μL (4.5 – 11.0) • RBC: 3.26 M/μL (4.6 – 6.2) • Plt: 3.16 x 1000/μL (150 – 400) • Hgb: 8.80 g/dL (14.5 – 18.1) • Hct: 26.7% (42 – 54) • MCV: 81.8 fL (80 – 100) • MCH: 27.0 pg (28 – 34) • RDW: 16.1% (11.5 – 14.5) • Abs Neutrophil: 1.63 x 1000/μL (1.8 – 7.8)
Case 2 • Differential diagnosis of pancytopenia with severe thrombocytopenia: • Marrow infiltrative process (leukemia, aplastic anaemia) • Immune Thrombocytopenic Purpura (ITP) • Why the neutropenia and anemia? • Next step, bone marrow biopsy.
Case 2 Diagnosis: • ITP • Iron deficiency anemia, secondary to menometrorrhagia from low platelets • Benign neutropenia
Case 2 • Patient received steroids, IVIG x1 • 1 week following, repeat CBC with a platelet count of 35
ITP Epidemiology of ITP: • Onset typically children 2-4 years
ITP • Differential diagnosis: • Diagnosis of exclusion, need to exclude drug-induced thrombocytopenia • Familial thrombocytopenia • Check family history of low platelets unresponsive to treatment for ITP • Thrombotic thrombocytopenic purpura (TTP) • Spurious thrombocytopenia resulting from platelet clumping • Always look at the peripheral smear of a patient with thrombocytopenia
ITP • Mechanism: • Patients platelets are coated with IgG antibodies. Tissue macrophages recognize the Fc receptor, and phagocytose. • May also have reduced megakaryocyte production.
ITP Diagnosis: • Diagnosis of exclusion • Should exclude other causes of thrombocytopenia • Review the peripheral smear • Bone marrow examination if atypical, if corticosteroid treatment is planned • Detection of anti-platelet antibodies(49-66% sensitive (not obligatory)
ITP Treatment: • IVIG • Steroids • Azathioprin (Imuran) • Splenectomy if refractory to treatment • TPO mimetics (p.o., s.c.) • Anti-D (????)
ITP Goal of treatment AVOID LIFE THREATENING BLEEDING