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Hematology/haematology is the branch of medicine concerned with the study of the cause, prognosis, treatment, and prevention of diseases related to blood. It involves treating diseases that affect the production of blood and its components, such as blood cells, hemoglobin, blood proteins, bone marrow, platelets, blood vessels, spleen, and the mechanism of coagulation. Such diseases might include hemophilia, blood clots, other bleeding disorders and blood cancers such as leukemia, multiple myeloma, and lymphoma. The laboratory work that goes into the study of blood is frequently performed by a medical technologist or medical laboratory scientist. Many hematologists work as hematologist-oncologists, also providing medical treatment for all types of cancer. The term is from the Greek αἷμα, haima meaning “blood,” and -λoγία meaning study.
Types of Hematology
Myeloid
Hemoglobinopathies (congenital abnormality of the hemoglobin molecule or of the rate of hemoglobin synthesis)
Sickle-cell disease
Thalassemia
Methemoglobinemia
Anemias (lack of red blood cells or hemoglobin)
Iron deficiency anemia
Megaloblastic anemia
Vitamin B12 deficiency
Pernicious anemia
Folate deficiency
Hemolytic anemias (destruction of red blood cells)
Immune mediated hemolytic anemia (direct Coombs test is positive)
Autoimmune hemolytic anemia
Warm antibody autoimmune hemolytic anemia
Idiopathic
Systemic lupus erythematosus (SLE)
Evans’ syndrome (antiplatelet antibodies and hemolytic antibodies)
Cold autoimmune hemolytic anemia
Cold agglutinin disease
Paroxysmal cold hemoglobinuria (rare)
Infectious mononucleosis
Alloimmune hemolytic anemia
Hemolytic disease of the newborn (HDN)
Rh disease (Rh D)
ABO hemolytic disease of the newborn
Anti-Kell hemolytic disease of the newborn
Rhesus c hemolytic disease of the newborn
Rhesus E hemolytic disease of the newborn
Other blood group incompatibility (RhC, Rhe, Kid, Duffy, MN, P and others)
Drug induced immune mediated hemolytic anemia
Penicillin (high dose)
Methyldopa
Hemoglobinopathies (where these is an unstable or crystalline hemoglobin)
Paroxysmal nocturnal hemoglobinuria (rare acquired clonal disorder of red blood cell surface proteins)
Direct physical damage to RBCs
Microangiopathic hemolytic anemia
Secondary to artificial heart valve(s)
Aplastic anemia
Fanconi anemia
Diamond-Blackfan anemia (inherited pure red cell aplasia)
Acquired pure red cell aplasia
Decreased numbers of cells
Myelodysplastic syndrome
Myelofibrosis
Neutropenia (decrease in the number of neutrophils)
Agranulocytosis
Glanzmann’s thrombasthenia
Thrombocytopenia (decrease in the number of platelets)
Idiopathic thrombocytopenic purpura (ITP)
Thrombotic thrombocytopenic purpura (TTP)
Heparin-induced thrombocytopenia (HIT)
Myeloproliferative disorders (Increased numbers of cells)
Polycythemia vera (increase in the number of cells in general)
Erythrocytosis (increase in the number of red blood cells)
Leukocytosis (increase in the number of white blood cells)
Thrombocytosis (increase in the number of platelets)
Myeloproliferative disorder
Coagulopathies (disorders of bleeding and coagulation)
Thrombocytosis
Recurrent thrombosis
Disseminated intravascular coagulation
Disorders of clotting proteins
Hemophilia
Hemophilia A
Hemophilia B (also known as Christmas disease)
Hemophilia C
Von Willebrand disease
Disseminated intravascular coagulation
Protein S deficiency
Antiphospholipid syndrome
Disorders of platelets
Thrombocytopenia
Glanzmann’s thrombasthenia
Wiskott-Aldrich syndrome
Hematological malignancies
Hematological malignancies
Lymphomas
Hodgkin’s disease
Non-Hodgkin’s lymphoma {includes the next five entries}
Burkitt’s lymphoma
Anaplastic large cell lymphoma
Splenic marginal zone lymphoma
Hepatosplenic T-cell lymphoma
Angioimmunoblastic T-cell lymphoma (AILT)
Myelomas
Multiple myeloma
Waldenström macroglobulinemia
Plasmacytoma
Leukemias increased WBC
Acute lymphocytic leukemia (ALL)
Chronic lymphocytic leukemia (CLL){now included in theCLL/SCLL type NHL}
Acute myelogenous leukemia (AML)
Chronic Idiopathic Myelofibrosis (MF)
Chronic myelogenous leukemia (CML)
T-cell prolymphocytic leukemia (T-PLL)
B-cell prolymphocytic leukemia (B-PLL)
Chronic neutrophilic leukemia (CNL)
Hairy cell leukemia (HCL)
T-cell large granular lymphocyte leukemia (T-LGL)
Aggressive NK-cell leukemia
Miscellaneous
Hemochromatosis
Asplenia
Hypersplenism
Gaucher’s disease
Monoclonal gammopathy of undetermined significance
Hemophagocytic lymphohistiocytosis
Tempi syndrome
Hematological changes secondary to non-hematological disorders
Anemia of chronic disease
Infectious mononucleosis
AIDS
Malaria
Leishmaniasis
Blood
Venous blood
Venipuncture
Hematopoiesis
Blood tests
Cord blood
Red blood cells
Erythropoiesis
Erythropoietin
Iron metabolism
Hemoglobin
Glycolysis
Pentose phosphate pathway
White blood cells
Platelets
Reticuloendothelial system
Bone marrow
Spleen
Liver
Lymphatic system
Blood transfusion
Blood plasma
Blood bank
Blood donors
Blood groups
Hemostasis
Coagulation
Vitamin K
Complement system
Immunoglobulins
(abnormality of the hemoglobin molecule or of the rate of hemoglobin synthesis)
Anemias (lack of red blood cells or hemoglobin)
Hematological malignancies
Coagulopathies (disorders of bleeding and coagulation)
Sickle Cell Anemia
Thalassemia
Evaluation
A 27 year-old male with sickle cell disease (HbSC) on hydroxurea and with a history of 2-3 hospitalizations per year for vaso-occlusive pain crises manifested by arthralgias and back pain presents to the emergency department with 3 days of worsening joint pain affecting his entire body but predominantly his knees and lower back. He is familiar with this pain and attempted therapy at home with ibuprofen, then hydrocodone-acetaminophen, and finally hydromorphone without improvement and presented to the emergency department.
On review of systems, he denied chest pain, cough, or shortness of breath. He has some periumbilical abdominal pain but tolerated normal oral intake on the day of presentation without vomiting nor changes in bowel habits. He otherwise denied fevers, peripheral numbness/weakness, urinary or fecal incontinence or retention. He similarly denies trauma, weight loss, night sweats, or intravenous drug use.
Objectively, the patient’s vital signs were normal and he was well-appearing. Mucous membranes were moist and skin turgor was normal. There were no appreciable joint effusions, warmth, nor limitation to active/passive range of motion of any joints. His back had no midline tenderness to palpation or percussion, normal range of motion in all axes and extremity sensation and strength testing were normal. Abdominal and genitourinary examinations were normal. The patient had preserved perineal sensation to light touch and normal rectal tone – a core temperature was obtained which was also normal.
Peripheral access was established and a parenteral dose of hydromorphone equivalent to his home oral dose was administered (0.015mg/kg). Repeat dosing was required at 15 minutes due to persistent pain scale of 10. Diphenhydramine and acetaminophen were also administered, for potential opioid-sparing effects, recognizing the limited evidence to support these relatively benign adjuncts.
Laboratory studies were notable for anemia (though stable compared to baseline measures), appropriate reticulocyte count, no evidence of hemolysis and with normal electrolytes and renal function.
A thorough history and examination did not identify a critical precipitant for the patient’s symptoms which were presumed to be secondary to a vaso-occlusive pain crisis. On reassessment, the patient’s pain was improved and an oral dose of hydromorphone was administered with continued observation and serial reassessments for two hours thereafter. The patient’s hematologist was available for follow-up the subsequent morning and the patient was discharged home.
Pharmacokinetics of Commonly-Used Opiate Analgesics
MEDICATION
ROUTE
ONSET
PEAK
DURATION
Morphine
IV
5-10min
20min
3-5h
IM
15-30min
30-60min
PO
30min
1h
Oxycodone
PO
10-15min
30-60min
3-6h
Hydrocodone
PO
10-20min
4-8h
Fentanyl
IV
<1min
2-5min
30-60min
Hydromorphone
IV
5min
10-20min
3-4h
PO
15-30min
30-60min
Codeine
PO
30-60min
60-90min
4-6h
Algorithm for the Evaluation and Management of Sickle Cell Crises
Chronic: anorexia, nausea/vomiting, constipation, fatigue, memory loss
Acute: CNS (lethargy, somnolence)
Findings
Calcium: >13.0mg/dL
ECG: QT shortening
Treatment
Mild: IVF
Severe: IVF, loop diuretics, bisophosphanate (pamidronate 90mg IV infused over 4 hours), consider calcitriol, consider hemodialysis if cannot tolerate fluids or unlikely to respond to diuretics
70M with a history of dementia presenting with 3 days of fatigue. The patient was unable to provide detailed history, however family members reported worsening fatigue with the patient requiring assistance with ambulation for several days. The patient was referred from an outside clinic after point-of-care hemoglobin of 6.7. No reported history of anemia, and no history suggestive of obvious external bleeding.
Vital signs stable, tachycardia and tachypnea noted with minimal exertion but saturating well on ambient air and in no acute distress. Examination notable for conjunctival pallor without scleral icterus, systolic flow murmur, brown stool guaiac negative.
CBC with hemoglobin of 7.5 , MCV 80.3 , RDW 22.4 , no leukocytosis and normal platelets. Also noted was an alkaline phosphatase of 828 , normal total and direct bilirubin, and undetectable serum troponin. Chest x-ray showed a possible pleural-based mass.
The patient was transfused two units of PRBC’s and admitted for further evaluation. CT chest/abdomen/pelvis revealed sternal and rib-based pleural soft-tissue mass, prostate mass, pelvic and retroperitoneal lymphadenopathy as well as extensive bony metastatic disease consistent with primary prostate cancer with diffused metastasis. Serum PSA was 2,087 . Iron studies suggested anemia of chronic disease. Reticulocytes were not obtained but may have suggested inadequate production index given extensive bony metastases and possible associated myelosuppression. The patient was symptomatically improved after transfusion and discharged with outpatient follow-up for discussions regarding possible biopsy and treatment.
Images
Areas of pleural thickening. Possible pleural based mass in left mid lung.
ct_chest_anemia_01
CT Chest: Lung Window
Rib-based pleural soft tissue masses.
Large 5.6 x 4.4cm anterior sternal soft-tissue mass.
ct_bone_anemia_01
CT Body: Bone Window
Extensive bony metastatic disease.
Prostate mass, large pelvic and retroperitoneal lymphadenopathy.
Consistent with primary prostate cancer with diffuse metastasis.
Algorithm for the Evaluation of Anemia
Lymphadenopathy Applied: Lymphoma
27 year-old female with no medical history presenting with neck swelling. She describes one month of progressive enlargement of a left-sided neck mass, and in the past two weeks has noted a new right-sided neck mass. This has been associated with worsening dysphagia to solids, describing a sensation of food lodging in the mid-chest and requiring liquids for passage – she attributes her recent 10lb weight loss to this. She also reports a non-productive cough for the past two weeks and generalized fatigue. She otherwise denies fevers, night sweats, chest pain, shortness of breath, nausea/vomiting, or changes in bowel/urinary habits. She has no known sick contacts or TB exposure risk factors. She has no medical history, no prior surgeries, does not take any medications and denies tobacco, alcohol or drug use.
Physical Exam
VS:
T
38.4
HR
98
RR
14
BP
108/68
O2
99% RA
Gen:
Well-appearing young female, in no acute distress.
HEENT:
PERRL, EOMI, MMM without lesions. There is a 2x3cm firm, non-tender, mobile left supraclavicular lymph node, as well as two 1x1cm firm, non-tender lymph nodes in the left and right anterior cervical chains.
CV:
RRR, normal S1/S2, no murmurs. No JVD.
Lungs:
Clear to auscultation bilaterally. There is a transition to bronchial breath sounds along the trachea inferior to the sternal angle with normal tracheal sounds superiorly.
Abd:
Soft, non-tender without organomegaly.
Ext:
Warm and well-perfused with normal peripheral pulses. No axillary or inguinal lymphadenopathy.
Neuro:
Alert and oriented, responding appropriately to questions. PERRL, EOMI, facial sensation symmetric, facial muscles symmetric, hearing grossly normal, palate rises symmetrically, tongue movements normal without fasciculation, SCM/trapezius normal. Normal FTN, RAM. Gait intact. Peripheral sensation and motor grossly normal.
Imaging
CT Chest – Axial
Anterior mediastinal mass with a wide differential – likely represents lymphoma or germ cell tumor. Less likely thymic or thyroid origin.
CT Chest – Sagittal
Anterior mediastinal mass with a wide differential – likely represents lymphoma or germ cell tumor. Less likely thymic or thyroid origin.
Assessment/Plan
27F with no PMH presenting with progressive localized lymphadenopathy. Resultant dysphagia, cervical and supraclavicular distribution as well as abnormal tracheal sounds concerning for mediastinal involvement. The patient is currently stable without evidence of airway compromise. ACT of the chest was obtained to evaluate for thoracic malignancy, which showed a large anterior mediastinal mass concerning lymphoma or germ cell tumor. The location of the mass likely explains the patient’s dysphagia due to compression of the esophagus, as well as the abnormal pulmonary exam with compression potentially irritating the trachea and triggering her non-productive cough. The patienthe t was admitted for further workup.
Lymphadenopathy Applied – Lymphoma
This case applies the differential diagnosis of lymphadenopathy. The most abnormal finding on examination was non-tender, left supraclavicular lymphadenopathy. The duration of symptoms and lack of tenderness is concerning for malignancy, and the left supraclavicular location suggests a thoracic or intra-abdominal source.
