NEET PG Exam > NEET PG Notes > Medicine > CheatSheet: Hematology
CheatSheet: Hematology
1. Anemia
1.1 Definition and Classification
| Parameter | Values |
|---|---|
| Hemoglobin (Adult Male) | <13 g/dL |
| Hemoglobin (Adult Female) | <12 g/dL |
| Hemoglobin (Pregnant Women) | <11 g/dL |
1.2 Morphological Classification
| Type | MCV (fL) |
|---|---|
| Microcytic | <80 |
| Normocytic | 80-100 |
| Macrocytic | >100 |
1.3 Iron Deficiency Anemia
| Feature | Finding |
|---|---|
| MCV | Decreased (<80 fL) |
| Serum Iron | Decreased |
| TIBC | Increased |
| Serum Ferritin | Decreased (<15 ng/mL) |
| Transferrin Saturation | Decreased (<15%) |
| Blood Film | Microcytic hypochromic, pencil cells, anisopoikilocytosis |
| First Depleted | Bone marrow iron stores |
| Most Sensitive Test | Serum ferritin |
1.4 Anemia of Chronic Disease
| Feature | Finding |
|---|---|
| Serum Iron | Decreased |
| TIBC | Decreased |
| Serum Ferritin | Normal or increased |
| Transferrin Saturation | Decreased |
| Key Mediator | Hepcidin (blocks iron release) |
1.5 Megaloblastic Anemia
| Vitamin B12 Deficiency | Folate Deficiency |
|---|---|
| Neurological manifestations present (SACD) | No neurological manifestations |
| Normal homocysteine, elevated MMA | Elevated homocysteine, normal MMA |
| Causes: Pernicious anemia, gastrectomy, terminal ileum disease | Causes: Malnutrition, pregnancy, alcoholism, drugs (methotrexate, phenytoin) |
| Schilling test positive in pernicious anemia | Red cell folate more specific than serum folate |
- Blood film: Macro-ovalocytes, hypersegmented neutrophils (>5 lobes)
- Bone marrow: Megaloblasts with nuclear-cytoplasmic asynchrony
- Pancytopenia may be present
- LDH and indirect bilirubin elevated (ineffective erythropoiesis)
1.6 Thalassemia
1.6.1 β-Thalassemia
| Type | Features |
|---|---|
| β-Thalassemia Major | Severe anemia (Hb 3-6 g/dL), transfusion dependent, hepatosplenomegaly, bone deformities, HbF elevated (>70%), HbA2 variable |
| β-Thalassemia Minor | Mild microcytic anemia, HbA2 elevated (>3.5%), target cells, basophilic stippling |
| HbE β-Thalassemia | Variable severity, HbE peak on HPLC |
1.6.2 α-Thalassemia
| Genes Deleted | Condition |
|---|---|
| 1 gene (-α/αα) | Silent carrier (asymptomatic) |
| 2 genes (-α/-α or --/αα) | α-Thalassemia trait (mild microcytic anemia) |
| 3 genes (--/-α) | HbH disease (moderate anemia, HbH on electrophoresis) |
| 4 genes (--/--) | Hydrops fetalis (incompatible with life, Hb Barts) |
1.7 Sickle Cell Disease
| Feature | Detail |
|---|---|
| Mutation | Glutamic acid → Valine at position 6 of β-globin |
| Blood Film | Sickle cells, target cells, Howell-Jolly bodies (hyposplenism) |
| Screening Test | Sickling test (sodium metabisulfite) |
| Confirmatory Test | Hemoglobin electrophoresis (HbS >50% in homozygous) |
| Crisis Types | Vaso-occlusive, aplastic, sequestration, hemolytic |
| Acute Chest Syndrome | Fever, chest pain, pulmonary infiltrates; leading cause of death |
| Treatment | Hydroxyurea (increases HbF), exchange transfusion for crisis |
1.8 Hemolytic Anemias
1.8.1 General Features
- Elevated reticulocyte count
- Elevated LDH and indirect bilirubin
- Decreased haptoglobin
- Elevated urobilinogen
- Hemoglobinuria in intravascular hemolysis
1.8.2 Hereditary Spherocytosis
| Feature | Detail |
|---|---|
| Defect | Spectrin/ankyrin deficiency (vertical interactions) |
| Blood Film | Spherocytes, increased MCHC |
| Osmotic Fragility | Increased |
| Direct Coombs | Negative |
| Treatment | Splenectomy curative |
1.8.3 G6PD Deficiency
| Feature | Detail |
|---|---|
| Inheritance | X-linked recessive |
| Triggers | Oxidant drugs (dapsone, primaquine, sulfonamides), fava beans, infections |
| Blood Film | Bite cells, Heinz bodies, blister cells |
| Type | Intravascular hemolysis |
1.8.4 Paroxysmal Nocturnal Hemoglobinuria
| Feature | Detail |
|---|---|
| Defect | PIGA gene mutation (CD55, CD59 deficiency) |
| Triad | Hemolytic anemia, pancytopenia, thrombosis |
| Diagnostic Test | Flow cytometry (absent CD55/CD59) |
| Treatment | Eculizumab (C5 inhibitor) |
1.8.5 Autoimmune Hemolytic Anemia
| Type | Features |
|---|---|
| Warm AIHA | IgG antibody, 37°C, extravascular hemolysis, causes: SLE, CLL, drugs (methyldopa) |
| Cold AIHA | IgM antibody, 4°C, intravascular hemolysis, causes: Mycoplasma, EBV, CLL |
| Direct Coombs | Positive in both (detects antibody on RBC) |
| Treatment | Steroids (warm), avoid cold exposure (cold), rituximab |
1.9 Aplastic Anemia
| Feature | Detail |
|---|---|
| Definition | Pancytopenia with hypocellular bone marrow (<25% cellularity) |
| Causes | Idiopathic (50%), drugs (chloramphenicol, NSAIDs), viral (hepatitis, EBV, parvovirus), radiation, Fanconi anemia |
| Bone Marrow | Hypocellular with fatty replacement |
| Treatment | Immunosuppression (ATG + cyclosporine) or bone marrow transplant |
| Growth Factor | Eltrombopag approved as adjunct |
1.10 Anemia in Pregnancy
- Physiological anemia: Plasma volume increases more than RBC mass
- Definition: Hemoglobin <11 g/dL
- Most common cause: Iron deficiency
- Folate requirement increases (5 mg/day supplementation)
2. Polycythemia
2.1 Definition and Classification
| Type | Features |
|---|---|
| Primary (Polycythemia Vera) | JAK2 mutation, increased RBC mass, low EPO |
| Secondary | Increased EPO (hypoxia, renal tumors, hepatoma), normal RBC mass relative |
| Relative | Decreased plasma volume (dehydration, stress) |
2.2 Polycythemia Vera
| Feature | Detail |
|---|---|
| Mutation | JAK2 V617F (95%), JAK2 exon 12 (3%) |
| Hemoglobin | >16.5 g/dL (male), >16 g/dL (female) |
| Hematocrit | >49% (male), >48% (female) |
| EPO Level | Low or normal |
| Clinical Features | Plethora, pruritus after bath, splenomegaly, thrombosis, erythromelalgia |
| Treatment | Phlebotomy (target Hct <45%), aspirin, hydroxyurea (high risk) |
| Complications | Thrombosis (major cause of death), transformation to AML or myelofibrosis |
3. Leukemia
3.1 Acute Lymphoblastic Leukemia
| Feature | Detail |
|---|---|
| Age | Peak at 2-5 years (most common childhood malignancy) |
| Clinical Features | Anemia, bleeding, infections, lymphadenopathy, hepatosplenomegaly |
| Blood Film | Lymphoblasts (>20%) |
| Immunophenotype | B-ALL (75%): CD19, CD10 (CALLA); T-ALL (25%): CD3, CD7 |
| Best Prognosis | t(12;21) TEL-AML1, age 2-10 years, WBC <50,000, hyperdiploidy |
| Worst Prognosis | t(9;22) Philadelphia chromosome, t(4;11) MLL gene, age <1 or >10 years, WBC >50,000 |
| CNS Prophylaxis | Intrathecal methotrexate mandatory |
| Treatment | Multi-agent chemotherapy, imatinib for Ph+ ALL |
3.2 Acute Myeloid Leukemia
| Feature | Detail |
|---|---|
| Age | Peak in adults (median 65 years) |
| Auer Rods | Pathognomonic for AML |
| Myeloperoxidase | Positive |
| M3 (APL) | t(15;17) PML-RARA, DIC, treated with ATRA + arsenic trioxide |
| M4/M5 | Monocytic leukemia, gum hypertrophy, skin infiltration |
| Best Prognosis | t(8;21), inv(16), t(15;17) APL |
| Worst Prognosis | Complex karyotype, monosomy 5/7, FLT3-ITD mutation |
| Treatment | Induction: 7+3 (cytarabine + daunorubicin), consolidation chemotherapy |
3.3 Chronic Myeloid Leukemia
| Feature | Detail |
|---|---|
| Genetic Defect | t(9;22) Philadelphia chromosome (BCR-ABL fusion) |
| Age | 40-60 years |
| Clinical Features | Massive splenomegaly, weight loss, hypermetabolism |
| Blood Film | Marked leukocytosis (WBC >100,000), entire myeloid spectrum, basophilia |
| LAP Score | Decreased (vs. leukemoid reaction where increased) |
| Phases | Chronic (85%, asymptomatic) → Accelerated → Blast crisis |
| Treatment | Imatinib (first-line TKI), dasatinib, nilotinib for resistance |
| Monitoring | BCR-ABL transcripts by RT-PCR |
3.4 Chronic Lymphocytic Leukemia
| Feature | Detail |
|---|---|
| Age | >60 years (most common adult leukemia in West) |
| Clinical Features | Asymptomatic lymphocytosis, lymphadenopathy, hepatosplenomegaly |
| Blood Film | Small mature lymphocytes, smudge cells (Gumprecht shadows) |
| Immunophenotype | CD5+, CD19+, CD20+, CD23+ |
| Diagnosis | Absolute lymphocyte count >5,000/μL for >3 months |
| Complications | Autoimmune hemolytic anemia, hypogammaglobulinemia, Richter transformation (DLBCL) |
| Treatment | Watch and wait (asymptomatic), FCR regimen, ibrutinib, venetoclax |
| Prognostic Markers | Good: del(13q), mutated IgVH; Poor: del(17p), del(11q), unmutated IgVH |
3.5 Hairy Cell Leukemia
| Feature | Detail |
|---|---|
| Blood Film | Hairy projections on lymphocytes |
| Staining | TRAP (tartrate-resistant acid phosphatase) positive |
| Immunophenotype | CD11c+, CD25+, CD103+ |
| Bone Marrow | Dry tap (fibrosis) |
| Clinical | Pancytopenia, massive splenomegaly |
| Treatment | Cladribine (2-CDA), excellent response |
4. Lymphoma
4.1 Hodgkin Lymphoma
| Feature | Detail |
|---|---|
| Characteristic Cell | Reed-Sternberg cells (CD15+, CD30+, CD20-) |
| Age Distribution | Bimodal (20-30 years and >55 years) |
| Clinical | Painless lymphadenopathy, B symptoms (fever, night sweats, weight loss >10%) |
| Alcohol-Induced Pain | Pathognomonic feature |
| Spread | Contiguous lymph node spread |
| Most Common Subtype | Nodular sclerosis (60-70%) |
| Best Prognosis | Lymphocyte-rich subtype |
| Worst Prognosis | Lymphocyte-depleted subtype |
| Staging | Ann Arbor staging system |
| Treatment | ABVD chemotherapy ± radiotherapy |
4.2 Non-Hodgkin Lymphoma
4.2.1 Diffuse Large B-Cell Lymphoma
| Feature | Detail |
|---|---|
| Frequency | Most common NHL (30%) |
| Clinical | Rapidly enlarging mass, extranodal involvement common |
| Immunophenotype | CD19+, CD20+ |
| Treatment | R-CHOP (rituximab + cyclophosphamide, doxorubicin, vincristine, prednisone) |
| Prognosis | IPI score (age, stage, LDH, performance status, extranodal sites) |
4.2.2 Follicular Lymphoma
| Feature | Detail |
|---|---|
| Genetic | t(14;18) BCL2 overexpression |
| Clinical | Painless lymphadenopathy, waxing and waning |
| Histology | Follicular architecture, centrocytes and centroblasts |
| Behavior | Indolent but incurable |
| Treatment | Watch and wait or rituximab-based therapy |
4.2.3 Burkitt Lymphoma
| Feature | Detail |
|---|---|
| Genetic | t(8;14) MYC translocation |
| Variants | Endemic (African, jaw mass, EBV+), sporadic (abdomen, ileo-cecal), immunodeficiency-related |
| Histology | Starry sky appearance (tingible body macrophages) |
| Immunophenotype | CD10+, CD20+, surface IgM+, Ki-67 ~100% |
| Treatment | Intensive chemotherapy with CNS prophylaxis |
4.2.4 Mantle Cell Lymphoma
| Feature | Detail |
|---|---|
| Genetic | t(11;14) Cyclin D1 overexpression |
| Immunophenotype | CD5+, CD20+, CD23- |
| Clinical | Aggressive, elderly males, multiple lymphomatous polyposis (GI) |
| Prognosis | Poor, median survival 3-5 years |
4.2.5 Marginal Zone Lymphoma
| Type | Features |
|---|---|
| MALT Lymphoma | Extranodal, gastric (H. pylori), t(11;18), responds to H. pylori eradication |
| Splenic Marginal Zone | Splenomegaly, villous lymphocytes |
| Nodal Marginal Zone | Lymph nodes, indolent |
4.2.6 Mycosis Fungoides
| Feature | Detail |
|---|---|
| Type | Cutaneous T-cell lymphoma |
| Clinical | Patches → plaques → tumors, Pautrier microabscesses |
| Sézary Syndrome | Leukemic variant, erythroderma, cerebriform nuclei |
| Treatment | Skin-directed therapy, phototherapy, systemic chemotherapy |
5. Myeloproliferative Neoplasms
5.1 Essential Thrombocythemia
| Feature | Detail |
|---|---|
| Platelet Count | >450,000/μL |
| Mutations | JAK2 V617F (50%), CALR (25%), MPL (5%) |
| Clinical | Thrombosis (arterial/venous), bleeding (acquired vWD), erythromelalgia |
| Blood Film | Large platelets, megakaryocyte fragments |
| Treatment | Aspirin, hydroxyurea (high risk), anagrelide |
5.2 Primary Myelofibrosis
| Feature | Detail |
|---|---|
| Mutations | JAK2 (50%), CALR (25%), MPL (10%) |
| Clinical | Massive splenomegaly, hepatomegaly, constitutional symptoms |
| Blood Film | Leukoerythroblastic picture, teardrop cells (dacrocytes) |
| Bone Marrow | Dry tap, collagen fibrosis (reticulin stain positive) |
| Treatment | Supportive, JAK inhibitors (ruxolitinib), stem cell transplant |
6. Plasma Cell Disorders
6.1 Multiple Myeloma
| Feature | Detail |
|---|---|
| Definition | Clonal plasma cells ≥10% in bone marrow + end-organ damage (CRAB) |
| CRAB Criteria | Calcium elevated, Renal insufficiency, Anemia, Bone lesions |
| M-Protein | IgG (60%), IgA (20%), light chain only (15-20%) |
| Bone Lesions | Lytic punched-out lesions (skull, vertebrae, ribs, pelvis) |
| Laboratory | Anemia, elevated calcium, elevated creatinine, rouleaux formation |
| Serum Protein | M-spike on electrophoresis |
| Urine | Bence Jones protein (light chains) |
| β2-Microglobulin | Prognostic marker (ISS staging) |
| Treatment | VRd (bortezomib, lenalidomide, dexamethasone), stem cell transplant |
| Complications | Hyperviscosity, AL amyloidosis, infections (encapsulated organisms) |
6.2 MGUS
| Feature | Detail |
|---|---|
| M-Protein | <3 g/dL |
| Plasma Cells | <10% in bone marrow |
| End-Organ Damage | Absent |
| Progression | 1% per year to myeloma |
| Management | Observation |
6.3 Waldenström Macroglobulinemia
| Feature | Detail |
|---|---|
| Cell Type | Lymphoplasmacytic lymphoma |
| M-Protein | IgM monoclonal protein |
| Mutation | MYD88 L265P (90%) |
| Clinical | Hyperviscosity syndrome (blurred vision, bleeding, neurologic), hepatosplenomegaly, lymphadenopathy |
| Treatment | Plasmapheresis (hyperviscosity), rituximab, bendamustine |
6.4 AL Amyloidosis
| Feature | Detail |
|---|---|
| Protein | Light chain deposition (λ > κ) |
| Organs Affected | Heart (most common cause of death), kidney (nephrotic syndrome), liver, tongue (macroglossia), peripheral nerves |
| Diagnosis | Tissue biopsy with Congo red stain (apple-green birefringence) |
| Treatment | Chemotherapy targeting plasma cells, stem cell transplant |
7. Bleeding Disorders
7.1 Platelet Disorders
7.1.1 Immune Thrombocytopenic Purpura
| Feature | Detail |
|---|---|
| Mechanism | Antiplatelet antibodies (IgG against GPIIb/IIIa) |
| Platelet Count | <100,000/μL |
| Bone Marrow | Increased megakaryocytes |
| Clinical | Petechiae, purpura, mucosal bleeding |
| Treatment | Steroids (first-line), IVIG (acute), rituximab, TPO agonists (romiplostim, eltrombopag), splenectomy |
| Life-Threatening | ICH (platelet count <10,000) |
7.1.2 Thrombotic Thrombocytopenic Purpura
| Feature | Detail |
|---|---|
| Mechanism | ADAMTS13 deficiency (cleaves vWF multimers) |
| Pentad | Thrombocytopenia, MAHA, fever, renal failure, neurologic symptoms |
| Blood Film | Schistocytes, elevated LDH |
| Coombs Test | Negative |
| Treatment | Urgent plasmapheresis, steroids, rituximab, caplacizumab (anti-vWF) |
7.1.3 Hemolytic Uremic Syndrome
| Feature | Detail |
|---|---|
| Triad | MAHA, thrombocytopenia, acute renal failure |
| Typical HUS | Diarrhea-associated (D+ HUS), Shiga toxin (E. coli O157:H7), children |
| Atypical HUS | Complement dysregulation, worse prognosis |
| Treatment | Supportive (typical), eculizumab (atypical) |
7.1.4 Heparin-Induced Thrombocytopenia
| Feature | Detail |
|---|---|
| Mechanism | Antibodies against PF4-heparin complex |
| Timing | 5-10 days after heparin exposure |
| Clinical | Thrombocytopenia + thrombosis (arterial/venous) |
| 4T Score | Predicts probability (Thrombocytopenia, Timing, Thrombosis, oTher causes) |
| Treatment | Stop heparin immediately, use argatroban or fondaparinux |
7.1.5 Bernard-Soulier Syndrome
| Feature | Detail |
|---|---|
| Defect | GPIb receptor deficiency (vWF binding) |
| Blood Film | Giant platelets |
| Bleeding Time | Prolonged |
| Platelet Aggregation | Absent response to ristocetin |
7.1.6 Glanzmann Thrombasthenia
| Feature | Detail |
|---|---|
| Defect | GPIIb/IIIa deficiency (fibrinogen binding) |
| Platelet Aggregation | Absent to all agonists except ristocetin |
| Bleeding Time | Prolonged |
7.2 Coagulation Disorders
7.2.1 Hemophilia A
| Feature | Detail |
|---|---|
| Defect | Factor VIII deficiency |
| Inheritance | X-linked recessive |
| PT | Normal |
| aPTT | Prolonged |
| Bleeding Time | Normal |
| Clinical | Hemarthrosis, muscle hematomas, delayed bleeding |
| Treatment | Factor VIII concentrate, desmopressin (mild cases) |
7.2.2 Hemophilia B
| Feature | Detail |
|---|---|
| Defect | Factor IX deficiency (Christmas disease) |
| Inheritance | X-linked recessive |
| Laboratory | Same as Hemophilia A (prolonged aPTT) |
| Treatment | Factor IX concentrate |
7.2.3 von Willebrand Disease
| Type | Features |
|---|---|
| Type 1 (75%) | Partial quantitative deficiency (autosomal dominant), mild bleeding |
| Type 2 | Qualitative defect (2A most common), variable bleeding |
| Type 3 | Complete deficiency (autosomal recessive), severe bleeding |
- vWF level: Decreased
- Factor VIII: Decreased (vWF carries Factor VIII)
- Bleeding time: Prolonged
- aPTT: Normal or prolonged
- Ristocetin cofactor assay: Decreased
- Treatment: Desmopressin (Type 1), vWF/Factor VIII concentrates
7.2.4 Disseminated Intravascular Coagulation
| Feature | Detail |
|---|---|
| Mechanism | Widespread thrombin generation and consumption of factors |
| Causes | Sepsis, trauma, malignancy (APL, mucin-secreting adenocarcinoma), obstetric (abruption, amniotic fluid embolism) |
| PT/aPTT | Prolonged |
| Platelet Count | Decreased |
| Fibrinogen | Decreased |
| D-Dimer | Markedly elevated |
| Blood Film | Schistocytes |
| Treatment | Treat underlying cause, FFP, cryoprecipitate, platelets |
7.2.5 Vitamin K Deficiency
| Feature | Detail |
|---|---|
| Factors Affected | II, VII, IX, X, Protein C, Protein S |
| PT | Prolonged (Factor VII has shortest half-life) |
| aPTT | Prolonged |
| Causes | Malabsorption, warfarin, broad-spectrum antibiotics, hemorrhagic disease of newborn |
| Treatment | Vitamin K, FFP (if active bleeding) |
8. Thrombotic Disorders
8.1 Inherited Thrombophilia
| Disorder | Features |
|---|---|
| Factor V Leiden | Most common (5% of population), resistance to activated Protein C, venous thrombosis |
| Prothrombin G20210A | 2nd most common, elevated prothrombin levels |
| Protein C Deficiency | Warfarin-induced skin necrosis, neonatal purpura fulminans |
| Protein S Deficiency | Similar to Protein C deficiency |
| Antithrombin Deficiency | Most thrombogenic, heparin resistance |
8.2 Acquired Thrombophilia
8.2.1 Antiphospholipid Syndrome
| Feature | Detail |
|---|---|
| Antibodies | Lupus anticoagulant, anticardiolipin, anti-β2 glycoprotein I |
| Clinical Criteria | Venous/arterial thrombosis, recurrent pregnancy loss |
| Laboratory | Prolonged aPTT (not corrected with mixing study), false-positive VDRL |
| Thrombocytopenia | Mild, common |
| Treatment | Lifelong anticoagulation, aspirin + LMWH in pregnancy |
9. Transfusion Medicine
9.1 Blood Components
| Component | Indication |
|---|---|
| Packed RBC | Hb <7 g/dL (restrictive), <8 g/dL in cardiac patients, acute blood loss |
| Platelets | <10,000 (prophylactic), <50,000 (surgery/bleeding), <100,000 (CNS surgery) |
| Fresh Frozen Plasma | Coagulation factor deficiency, warfarin reversal, DIC, TTP |
| Cryoprecipitate | Fibrinogen <100 mg/dL, hemophilia A, vWD, uremic bleeding |
9.2 Transfusion Reactions
| Type | Features |
|---|---|
| Acute Hemolytic | ABO incompatibility, fever, flank pain, hemoglobinuria, DIC, within minutes-hours |
| Delayed Hemolytic | Anamnestic response, 2-14 days post-transfusion, mild jaundice |
| Febrile Non-Hemolytic | Most common, anti-leukocyte antibodies, fever/chills within 1-6 hours, treat with antipyretics |
| Allergic | Urticaria, pruritus, treat with antihistamines |
| Anaphylactic | IgA deficiency, hypotension, bronchospasm, treat with epinephrine |
| TRALI | Bilateral pulmonary infiltrates within 6 hours, hypoxia, non-cardiogenic, supportive care |
| TACO | Volume overload, dyspnea, JVP elevated, pulmonary edema, diuretics |
9.3 Blood Group Systems
| Blood Group | Antibody |
|---|---|
| A | Anti-B |
| B | Anti-A |
| AB (Universal Recipient) | None |
| O (Universal Donor) | Anti-A and Anti-B |
- Rh-negative patients should receive Rh-negative blood
- Rh-positive patients can receive Rh-positive or Rh-negative blood
- Anti-D prophylaxis (RhoGAM) given to Rh-negative mothers
- Coombs test: Direct (antibody on RBC), Indirect (antibody in serum)
10. Miscellaneous Disorders
10.1 Porphyrias
| Type | Features |
|---|---|
| Acute Intermittent Porphyria | Autosomal dominant, PBG deaminase deficiency, abdominal pain, neuropsychiatric, port-wine urine, precipitated by drugs (CYP450 inducers) |
| Porphyria Cutanea Tarda | Most common, uroporphyrinogen decarboxylase deficiency, photosensitivity, skin blistering, hypertrichosis, associated with HCV, alcohol |
| Erythropoietic Protoporphyria | Ferrochelatase deficiency, photosensitivity in childhood, liver disease |
10.2 Methemoglobinemia
| Feature | Detail |
|---|---|
| Mechanism | Fe2+ oxidized to Fe3+ (cannot bind oxygen) |
| Causes | Dapsone, nitrates, local anesthetics (benzocaine), aniline dyes |
| Clinical | Cyanosis (chocolate-brown blood), normal PaO2, low oxygen saturation by pulse oximetry |
| Treatment | Methylene blue (activates NADPH methemoglobin reductase) |
10.3 Eosinophilia
| Cause | Examples |
|---|---|
| Parasitic Infections | Tissue-invading helminths (Ascaris, hookworm, Strongyloides, filariasis) |
| Allergic Disorders | Asthma, allergic rhinitis, drug reactions |
| Malignancy | Hodgkin lymphoma, T-cell lymphoma, hypereosinophilic syndrome |
| Vasculitis | Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) |
| Other | Addison disease, cholesterol emboli, adrenal insufficiency |
10.4 Neutropenia
| ANC (cells/μL) | Classification |
|---|---|
| 1500-1800 | Mild |
| 1000-1500 | Moderate |
| <500 | Severe (high infection risk) |
- Causes: Chemotherapy, drugs (clozapine, carbimazole), aplastic anemia, megaloblastic anemia, infections (typhoid, brucellosis)
- Cyclic neutropenia: Every 21 days
- Felty syndrome: RA + splenomegaly + neutropenia
- Treatment: G-CSF (filgrastim), antibiotics for febrile neutropenia
10.5 Leukemoid Reaction
| Feature | Leukemoid Reaction |
|---|---|
| WBC Count | >50,000/μL |
| LAP Score | Increased (vs. CML where decreased) |
| Philadelphia Chromosome | Absent |
| Basophilia | Absent |
| Causes | Severe infection, hemorrhage, hemolysis, malignancy |
10.6 Hypersplenism
- Pancytopenia due to splenic sequestration
- Splenomegaly present
- Bone marrow normal or hyperplastic
- Correction after splenectomy
- Causes: Portal hypertension, storage diseases, infections (malaria, kala-azar), lymphoproliferative disorders
10.7 Post-Splenectomy Changes
- Howell-Jolly bodies (nuclear remnants)
- Target cells
- Acanthocytes
- Thrombocytosis
- Increased infection risk: Encapsulated organisms (Streptococcus pneumoniae, Haemophilus influenzae, Neisseria meningitidis)
- Vaccination required: Pneumococcal, Hib, meningococcal (2 weeks before splenectomy)
- Prophylactic penicillin lifelong
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