Hematological Conditions: Diagnosis and Treatment

• The document provides an overview of hematological conditions, their diagnostics, etiologies, and potential treatments.
• It is intended to be a quick reference guide for healthcare professionals involved in the diagnosis and management of hematological disorders.
• It includes acute hematology, anemia, hemostasis, and transplantations.

Acute Hematology

• Always consider acute leukemia when blasts are present in the blood, or when there is a penil or proliferative condition.

Hæmopoiese

• Illustrates the differentiation of hematopoietic stem cells into different cell types

V. cava Superior Syndrome

• Occurs when expanding tumor masses in the mediastinum compress the superior vena cava which hinders venous return to the heart, causing venous stasis.
• Can be caused by thymoma, teratoma, thyroid cancer, and terrible lymphoma, known as the "4 Ts."
• Manifests as increasing difficulty breathing, facial swelling especially around the eyes, nasal congestion, vision disturbances, headache, dizziness, swelling of arms and neck, and prominent veins in the upper chest, neck, and arms.

Tumor Lysis

• This occurs when a large number of tumor cells break down, releasing intracellular components such as urate, phosphate, and potassium.
• Can lead to hyperuricemia, potentially causing acute renal failure.
• Commonly seen in rapidly growing lymphomas and leukemias, particularly after the commencement of chemotherapy for acute leukemias.
• Remember to check blood samples for elevated phosphate, urate, creatinine, and potassium, and decreased calcium.
• Treatment involves hydration, allopurinol/rasburicase to promote urate excretion, and bicarbonate.

Medullar Tversnittsyndrom

• Primarily seen with myelomatosis and lymphomas in hematology.
• Presents with pain and progressive deficits, such as impaired gait/paresis, sensory loss, bladder paresis, urinary retention, and anal sphincter involvement.
• Caused by tumor pressure or vertebral collapse with protrusion into the spinal medulla.
• Diagnosis through acute MR of the columna and CT scans.
• Acute treatment includes corticosteroids, radiation therapy, or surgery.

Leukostase

• Characterized by an excessive number of cells in the blood, which impairs blood flow.

APL - Akutt Promyelocytisk Leukæmi

• It is a type of acute myeloid leukemia with a high risk of bleeding needs prompt recognition and treatment.
• With timely treatment, the 5-year survival rate exceeds 90%; most deaths occur within the first month if not treated promptly.
• Classified under AML.

Infeksjoner Hos Immunsvekkede

• This section addresses infections in immunocompromised patients.
• Characterized by febril neutropeni.
• Benmargsinsuffisiens can be caused by vitamin B12 and folic acid deficiencies, aplastic anemia, metastases, splenomegaly, or rheumatologic conditions.
• Can also be medicine related such as Cytostatics, NSIA, and AB.
• The risk of infections increases exponentially when neutrophil counts drop below 0.5 x 10^9/L.
• Opportunistic infections, such as fungal infections, risk increases when having it over 7 days period.
• Treatment initates AB with coverage of tazocin.

Anemia

• Defined as a reduced erythrocyte mass in the body.
• It is caused by blood loss, impaired production, or hemolysis
• Blood loss can be acute or chronic and may affect iron metabolism.
• Impaired production can result from hypoplastic bone marrow like megaloblastic anemias.
• Hemolysis can be extra- or intra-erythrocytic
• Red blood cell kinetic studies involve assessment using reticulocytes
  • • reticulocytosis: blood loss and hemolysis
  • Normal/low reticulocytes: low production in bone marrow

Mikrocytær Anæmi

• Common causes include iron deficiency due to chronic bleeding, insufficient dietary intake, pregnancy, breastfeeding, and malabsorption.
• A long process leads to anemia, so check ferritin, and perform blood and plasma exams.
• Iron supplementation is administered, and blood transfusions may be necessary for severe cases.

Normocytær Anæmi

• Pathogenesis involves IL-6, which increases hepcidin production, reduces iron uptake from the intestine, reduces iron release from macrophages, reduces erythrocyte lifespan and EPO production, and reduces EPO sensitivity.

Makrocytær Anæmi

• Is from low reticulocyte count or hypoproliferative.
• B12: normal cost 5-7 mg/day, but you need 2,5 mg = 2-3 years consumption.
  • Caused by insufficient intake, pernicious anemia (lack of IF --> poor absorption of B12)
  • Finding: low B12, elevated hemocystine, elevated methylmalonate
• Folat: normal cost: 100-500 µg/day, but you need 100, a depot of 3 mdr.
  • Can be can by absorption (celiac, mb. crohns, gut, pregnancy etc.)
  • Finding: Low Folat, homocystein elevated, methylmalonate normal.

Hemolyse

• It is a Nedset erythrocytlevetid, which can lead to hemolytisk anæmi, so hemolyse + anemi
• Extravascular vs Intravascular hemolyse:
  • intravskulær testing: LDH high, Billirubin normal, fritt hbg hight, hemoglobinuri
    • Causes: Mikroangiopatisk (TTP, HUS, hjertklapp) Transfusjonkomplkasjoner Infeksjoner paroksysmal nokturna Kuldeagglutinsykdom
• Ekstravaskulær: Intrakorpsuklære: hemoglobinopatier, Membranopatier, Enzymopatier

Autoimmun Hemolytiske Anemi

• Anemia caused by autoantibodies that react with red blood cell membrane antigens.
• Diagnose via the DAT Coombs test.
• Treatment-
  • Predisolone.
  • Rituximab.
  • Splenoektomi.

Membranopatier

• Hereditary spherocytosis: genetic.
• Clinically: Hemolytic anemia. Splenomegaly. Icterus.
• Diagnose via Ektacytometri & EMA test.
• Treatment involves speleoctomi.

Enzymopatier: G6PD mangel

• Anemia aused by gentic mutation.
• Diagnosis is via erythrocytter measureent.
• Is utbredd where malaria is.
• Diagnosis often includes hemoanalyse aktivitet.

Hemoglobinopatier

• Is the most common genetic condition in the world.

• Divided into -Thalassæmi: quantitative defect of hemoglobin. Inndeles efter om det er alfa eller beta globina som ikke can formed.

• Alfa - Thalssemi: beta-globin > alfa-globina --> HbH (beta-tetramer) Forms.

• beta-thalasssemi: Alfa-globin > beta-globin --> a-globin hgb--> Insuffisient erythropiesis, mikrocytisk anemi og hemolyse. - Inndeling

• minor - heterozygot.

• Intermedia- thalassæmi genvarienater fra begge foreldre.

• Major: thalassmic genvariant fra begge ford --> fast transfuson

    --Treatment often includes:
  

• Blod-transfusion.

• Benmargs transfusion- try to live with.

Hemostase

• Assessment includes the duration of symptoms, the types of bleeding, and potential underlying causes
• Biochemical evaluation:
  • b-trombocytter
  • APTT (activated partial tromboplastin time)
  • P-koagulasjonsfaktorer
  • P-fibrin-D-dimer.

Blødningstendens

• Inquire for blødningensted via (ISH self, BAT).
• Type 0 bleeds more than type A/B.
• Numbers of bleeding in normal ranges:
  • Under 12 year old women
  • Under 12 year old men
  • Women above 12 years. -Men about 12 years old.
• Always look for more that one bleeding symptom.

Uforklarelig Alvorlig Blødning

• Check patient familial history.
• Check family bleeding history.
• Check for surgery.

Paraklinsick Utredning

• Check if Patient is taking trombocytt, INR, APTT,

ITP is immun trombocytopeni

• Patient is often newly-diagnosed from hud eller slimhinner
• Is ofte upåvriket.
• Often patient exhibits trombocytpenia and ingen andre tegn for sygdom

Hemofili- often X-Bundne Sygdom.

• Ofte from Men are more than men.
• There is often a Defekt i VIII - hemofill A
• There is often Defekt i IX - hemfill B.
• From there there are many incidents often spontane.
• Treatment often includes profylase.

Trombofili

*Trombofili- tendens for blodpropp- often men og krop can lead to venos thromboemboli

• Is for people with immobilisation. Hjertesvigt. * Graviditet Risk of Trombose is STASE
• There are many things with ENDOTELISK.

Genetics and RISIKO FAKTOR

• Hvom trombolfili, Often patient is under 50 ar.

DIC- intravaskulær koagulasjon

• DIC is the akut tilstand med akut utbredt aktivering av homostase og utbredte tromboser
• Is charcterizarer for Akut.
• Patients use many trompocyrer of other related factors.
• Causes- Ofest infeksjoner, meget tilskadekomende.
• Ved nogen typer kretf- * often nedsatt trombocytter eller fibrinogen Forlan.
• Often patients are often behandle grunnsykdomer

Allogen and Hematopoietisk cells transplantasjon

• Allo-HCT er Overforesel av bloddannede stamceller fra en dontor til recipienten og can often lead to other sygdoms then is Snakk om hoy-risiko behaundling
• Pasient-Faktoree-- - Skal kunnemedvirke til behandling and kan funksjonere under behandling
• Donors ofte often look etter - HLA. and there are often other genetic factors when the factor skiller mellem selv og Ikke-Selv
• To check for these factors- man look etter for ALLLELER
• Often the best choice is often HLA
• Kjemoterapi- formlaet- er orbygge evtoing. . -AB proflase
• Patient often are also have darligl iggeimmunforsvar under behandlong