Types[edit]

By affected component[edit]

 Humoral immune deficiency, with signs or symptoms depending on the cause, but generally
include signs ofhypogammaglobulinemia (decrease of one or more types of antibodies) with
presentations including repeated mildrespiratory infections,
and/or agammaglobulinemia (lack of all or most antibody production) which results in
frequent severe infections and is often fatal.[1]
 T cell deficiency, often causes secondary disorders such as acquired immune deficiency
syndrome.[2]
 Granulocyte deficiency, including decreased numbers of granulocytes (called
as granulocytopenia or, if absent,agranulocytosis) such as of neutrophil
granulocytes (termed neutropenia). Granulocyte deficiencies also include decreased
function of individual granulocytes, such as in chronic granulomatous disease.
 Asplenia, where there is no function of the spleen
 Complement deficiency is where the function of the complement system is deficient

n reality, immunodeficiency often affects multiple components, with notable examples

including severe combined immunodeficiency (which is primary) and acquired immune
deficiency syndrome (which is secondary).
 Comparison of immunodeficiencies by affected component

Affected Main pathogens of resultant

Main causes[3]
components infections[3]

 Primary humoral  Streptococcus pneumoniae

Humoral B cells, plasma  Multiple myeloma  Hemophilus influenzae
immune cells orantibodie  Chronic lymphoid  Pneumocystis jirovecii
deficiency s leukemia  Giardia intestinalis
 AIDS  Cryptosporidium parvum

 Marrow and
othertransplantation Intracellular pathogens,
T cell  AIDS including Herpes simplex
T cells
deficiency  Cancer chemotherapy virus,Mycobacterium, Listeria,[4] an
 Lymphoma d intracellular fungal infections.[3]
 Glucocorticoid therapy

 Chemotherapy
 Enterobacteriaceae
 Bone marrow
 Oral Streptococci
transplantation
Neutrophil  Pseudomonas aeruginosa
Neutropenia  Dysfunction, such
granulocytes  Enterococcus species
aschronic
 Candida species
granulomatous
 Aspergillus species
disease

 Polysaccharide encapsulated
bacteria,[5] particularly:
 Splenectomy  Streptococcus
Asplenia Spleen  Trauma pneumoniae[5]
 Sickle-cell anemia  Haemophilus influenzae[5]
 Neisseria meningitidis[5]
 Plasmodium species
  Babesia species

Complemen Complement  Congenital  Neisseria species

t deficiency system deficiencies  Streptococcus pneumoniae
Primary or secondary[edit]

Distinction between primary versus secondary immunodeficiencies are based on, respectively,
whether the cause originates in the immune system itself or is, in turn, due to insufficiency of a
supporting component of it or an external decreasing factor of it.

Primary immunodeficiency (PID)[edit]

Main article: Primary immunodeficiency

A number of rare diseases feature a heightened susceptibility to infections from childhood

onward. Primary Immunodeficiency is also known as congenital immunodeficiencies.[6] Many of
these disorders are hereditary and areautosomal recessive or X-linked. There are over 80
recognised primary immunodeficiency syndromes; they are generally grouped by the part of the
immune system that is malfunctioning, such as lymphocytes or granulocytes.[7]

The treatment of primary immunodeficiencies depends on the nature of the defect, and may
involve antibody infusions, long-term antibiotics and (in some cases) stem cell transplantation.

Secondary immunodeficiencies[edit]
Further information: Immunosuppression

Secondary immunodeficiencies, also known as acquired immunodeficiencies, can result from

various immunosuppressiveagents, for example, malnutrition, aging and particular medications
(e.g. chemotherapy, disease-modifying antirheumatic drugs, immunosuppressive
drugs after organ transplants, glucocorticoids). For medications, the
term immunosuppressiongenerally refers to both beneficial and potential adverse effects of
decreasing the function of the immune system, while the term immunodeficiency generally
refers solely to the adverse effect of increased risk for infection.

Many specific diseases directly or indirectly cause immunosuppression. This includes many
types of cancer, particularly those of the bone marrow and blood cells
(leukemia, lymphoma, multiple myeloma), and certain chronic infections. Immunodeficiency is
also the hallmark of acquired immunodeficiency syndrome (AIDS),[6] caused by the human
immunodeficiency virus (HIV). HIV directly infects a small number of T helper cells, and also
impairs other immune system responses indirectly.
Immunodeficiency and human immunodeficiency virus (HIV)
Immunodeficiency of genetic origin is called primary immunodeficiency.
If it is due to some other cause, it is secondary immunodeficiency.
Primary immunodeficiencies are rare and are usually due to a defect in a
single gene. They include defects in phagocyte function, defects in
complement, and defects of lymphocyte function. In all cases, there is
an increased susceptibility to infection.
Secondary immunodeficiency is far more common, particularly in adults.
The most common cause is malnutrition, but damage to the immune
system by certain infections (notably HIV—see below), tumors,
traumas, and some medical interventions also impair the function of the
immune system (e.g. treatment with immunosuppressive drugs or
exposure of the bone marrow to high levels of X -irradiation).
Summary

1. The immune system may react powerfully to an antigen

(hypersensitivity) or it may fail to mount an adequate immune
response (immunodeficiency). Host cells are not normally attacked
by the cells of the immune system, which are able to differentiate
self from non-self.
2. Hypersensitive reactions may be grouped under one of four
headings:
a. Allergic reactions, e.g. hay fever and asthma.
b. Cytotoxic hypersensitivity, which may occur after a tissue
transplant or when the lymphocytes attack the host cells
themselves (autoimmunity).
c. The deposition of immune complexes in small blood vessels,
leading to an inappropriate inflammatory reaction (e.g .
glomerulonephritis).
d. Cell-mediated hypersensitivity—a delayed allergic reaction
that follows exposure to certain antigens.
3. Immunodeficiency may be of genetic origin (primary
immunodeficiency) or due to some other cause (secondary
immunodeficiency). Primary immunodeficiencies are usually due to
a defect in a single gene. Secondary immunodeficiency is more
common and may result from malnutrition or from damage to the
immune system caused by certain infections, tumors, traumas, or
some medical treatments.
The human immunodeficiency virus (HIV) is perhaps the best known
infectious agent that can compromise the function of the immune
system. Unlike other agents, HIV attacks the cells of the immune
system, particularly the T cells. The explanation for this ala rming state
of affairs is that the T cell receptor is also the receptor to which HIV
binds. HIV is a retrovirus that can insert its genetic material into the
host cell DNA. Therefore the stimulation of an infected T cell results in
replication of the virus. This leads to a slow depletion of the T cell
population and an increased susceptibility to infection. The resulting
disease is now known as acquired immunodeficiency syndrome (AIDS).
14.7 Transplantation and the immune system
Tissue transplantation from one person to another to treat organ failure
has long been a major goal of medicine. The first such procedure to be
wholly successful was the transfusion of blood. This depended on the
correct identification of the agglutinins (antibodies) and agglutinogen s
(antigens) present in both donor and recipient blood as described
in Chapter 13, p. 243. In the same way, successful skin grafts or organ
transplants require a close match between the specific cell markers of
both donor and recipient.
The problems associated with transplantation are very well illustrated by
the difficulties experienced in successfully grafting skin. If the skin
becomes severely damaged, for example as a
P.259

result of extensive burns, the healing process cannot make good the lost
germinal tissue and the wound contracts as it becomes infiltrated by
connective tissue. This results in disfigurement and distortion of the
neighboring tissue. If the affected area is large, there may also be a
continued loss of fluid from the damaged area, whic h will also be
susceptible to infection. For these reasons it is sometimes desirable to
graft some healthy skin from another part of the body onto the site of
injury. Such grafts (which are known as autografts) are usually
successful. The transplanted tiss ue is quickly infiltrated by blood vessels
and heals into place. The donor area also heals rapidly. However, if the
damaged area is very extensive, it may be impossible to find sufficient
undamaged skin to act as a source for the grafts. In this case, it i s
necessary to consider grafting skin from someone else—to use
an allograft.
A skin graft from another individual will initially take quite well, but
after about a week it will be rejected. Moreover, a second graft from the
same donor will be rejected im mediately. However, if the donor is an
identical twin, the first graft will not be rejected as both twins have the
same genetic make -up and their tissue antigens (MHC or, in humans,
HLA) will be identical. These discoveries were crucial evidence that
tissue rejection is an immunological phenomenon and paved the way for
successful skin grafts and organ transplants between individuals of
different genetic backgrounds.
The solution to graft rejection is to match the tissue of the donor as
closely as possible with that of the recipient (tissue typing) and to
inhibit the activity of the immune system with immunosuppressant
drugs. This approach has proved very successful with skin grafts and
with kidney and heart transplants. More than 80 per cent of kidney
transplants will survive for more than 5 years provided that the HLA is
well matched. For heart transplants the figure is more than 70 per cent.
The success rate for the transplantation of other organs is also showing
considerable improvement. Nearly half of all liver transplant patients will
survive for more than 5 years, although lung transplants are less
successful at present. Close tissue matching is not required for corneal
grafts because the cornea does not become vascularized and so is not
subject to attack by the lymphocytes.

Causes of immunodeficiency commonly encountered are:

 Malignancies (Leukemias, lymphomas, Hodgkin's Disease).

In one study of cancer patients, fungal septicemia and pneumonias accounted for almost a third of deaths.
 Drug therapies. Anti-neoplastic substances, steroids, immunosuppressive drugs.
 Antibiotics. Over-use or inappropriate use of antibiotics can also contribute to the development of fungal
infections by altering the normal flora of the host and facilitating fungal overgrowth or by selecting for
resistant organisms.
 FUNGAL CENTRAL NERVOUS
SYSTEM INFECTION

PATHOGENESIS

•Mucosal colonization blood stream

spread in the CSF blood-brain barrier

•High number of microorganisms local energy

metabolism switch to anaerobic glycolysis

lactate concentration, CSF glucose

 • Inflammation local irritation abnormal
neuronal
activity
• Pus collection, subsequent fibrosis around brainstem
interference with cranial nerve function
• Focal pus collection (brain abscess)
intracranial pressure
• Different microorganisms are more likely to cause
CNS infection in patients with immunocompromised
defence e.g Cryptococus neoformans

FUNGAL INFECTIONS THAT MAY BE MANIFEST AS

CHRONIC MENINGITIS OR BRAIN ABSCESS

Cryptococcus neoformans
Coccidioides immitis
Histoplasma capsulatum
Candida albicans
Blastomyces dermatitidis*
* Disease manifest as brain abscess

MENIGITIS

Meningitis adalah infeksi meninges yang membran yang menutupi otak dan

sumsum tulang belakang.

Apakah meninges?

Meninges adalah 3 lapisan jaringan ikat. Mereka terdiri dari pia mater (terdekat ke

organ-organ sistem saraf pusat), arachnoid dan dura mater (terjauh dari otak dan

sumsum tulang belakang).

Mereka juga termasuk pembuluh darah dan berisi Cairan serebrospinal. Ini adalah

struktur yang terlibat dalam meningitis, peradangan meninges, yang, jika parah,

mungkin menjadi ensefalitis, radang otak.

Apa itu meningitis?

Meningitis adalah infeksi meninges. Infeksi dapat disebabkan oleh bakteri atau

virus, dan mengarah ke meninges menjadi meradang (bengkak). Ini dapat

menyebabkan kerusakan serius pada saraf, otak dan sumsum tulang belakang.

Gejala meningitis

Meningitis umumnya diwujudkan oleh:

 sakit kepala parah

 muntah

 demam tinggi

 kekakuan leher

 sensitivitas dan mata sakit pada paparan cahaya

 ruam kulit

Bakterial[sunting | sunting sumber]

Jenis bakteri penyebab meningitis bakterial bervariasi sesuai kelompok usia individu yang

terinfeksi.

 Pada bayi prematur dan anak baru lahir berusia hingga tiga bulan, penyebab yang sering
adalah streptokokus grup B (subtipe III yang biasanya hidup di vagina dan terutama
merupakan penyebab pada minggu pertama kehidupan) dan bakteri yang biasanya hidup
dalam saluran pencernaan seperti Escherichia coli (membawa antigen K1). Listeria
monocytogenes (serotipe IVb) dapat mengenai bayi baru lahir dan menimbulkan epidemi.
 Pada anak yang lebih besar seringkali disebabkan oleh Neisseria
meningitidis (meningokokus) dan Streptococcus pneumoniae (serotipe 6, 9, 14, 18, dan 23)
dan untuk balita oleh Haemophilus influenzae type B (di negara-negara yang tidak
memberikan vaksinasi).[1][3]
 Pada orang dewasa, Neisseria meningitidis dan Streptococcus pneumoniae merupakan
penyebab 80% kasus meningitis bakterial. Risiko terinfeksi oleh Listeria
monocytogenes meningkat pada orang yang berusia di atas 50 tahun.[3][4] Pemberian vaksin
pneumokokus telah menurunkan angka meningitis pneumokokus pada anak dan dewasa.[12]
Virus[sunting | sunting sumber]

Berbagai virus penyebab meningitis mencakup enterovirus, virus Herpes simpleks tipe 2 (dan
yang lebih jarang tipe 1), virus Varicella zoster (dikenal sebagai penyebab cacar airdan cacar
ular), paromiksovirus, HIV, dan LCMV
Jamur[sunting | sunting sumber]

Beberapa faktor risiko untuk meningitis jamur, antara lain penggunaan obat

imunosupresan (misalnya setelah transplantasi organ), HIV/AIDS,[16] dan hilangnya kekebalan

yang berhubungan dengan penuaan.[17] Hal ini jarang dijumpai pada orang dengan sistem

kekebalan tubuh normal[18] tetapi telah muncul karena kontaminasi obat.[19]Gejala awal biasanya

lebih gradual, dengan adanya sakit kepala dan demam selama setidaknya dua minggu sebelum

diagnosis ditegakkan.[17] Meningitis jamur yang paling sering adalahmeningitis

cryptococcal akibat Cryptococcus neoformans.[20] Di Afrika, meningitis cryptococcal diperkirakan

merupakan penyebab meningitis yang paling sering dijumpai [21] dan ini mencakup 20–25%

kematian yang berhubungan dengan AIDS di Afrika.[22] Jenis jamur lain yang sering dijumpai

adalah spesies Histoplasma capsulatum, Coccidioides immitis,Blastomyces dermatitidis,

dan Candida.
Penyebab
Meningitis jamur langka dan biasanya merupakan hasil dari penyebaran jamur
melalui darah ke sumsum tulang belakang.Meskipun setiap orang bisa
mendapatkan meningitis jamur, orang dengan sistem kekebalan tubuh yang lemah,
seperti orang-orang dengan infeksi HIV atau kanker, berada pada risiko yang lebih
tinggi.
Penyebab paling umum dari meningitis jamur untuk orang-orang dengan sistem
kekebalan yang lemah adalah Cryptococcus . Penyakit ini adalah salah satu
penyebab paling umum dari meningitis dewasa di Afrika.
Transmisi
Meningitis jamur tidak menular , yang berarti tidak ditularkan dari orang ke
orang. Meningitis jamur dapat berkembang setelah jamur menyebar melalui aliran
darah dari tempat lain dalam tubuh, sebagai akibat dari jamur yang diperkenalkan
langsung ke dalam sistem saraf pusat, atau dari infeksi situs tubuh yang terinfeksi
samping sistem saraf pusat.
Investigasi jamur Meningitis 2012
Pada bulan September 2012, Pusat Pengendalian dan Pencegahan Penyakit (CDC),
bekerja sama dengan departemen kesehatan negara bagian dan lokal dan Food and
Drug Administration (FDA), mulai menyelidiki wabah multistate meningitis jamur
dan infeksi lainnya di antara pasien yang menerima pengawet terkontaminasi
suntikan steroid bebas MPA dari New England Compounding Center di Framingham,
Massachusetts.
Untuk informasi lebih lanjut, lihat
 Wabah Multistate dari jamur Meningitis dan Infeksi Lain
 Sumber daya untuk Pasien
 Sumber daya untuk Dokter
 Sumber daya untuk Laboratorium
Anda juga bisa mendapatkan meningitis jamur setelah mengambil obat yang
melemahkan sistem kekebalan tubuh . Contoh obat ini termasuk steroid (seperti
prednisone), obat-obatan diberikan setelah transplantasi organ, atau obat anti-TNF,
yang kadang-kadang diberikan untuk pengobatan rheumatoid arthritis atau kondisi
autoimun lainnya.
Berbagai jenis jamur yang ditransmisikan dalam beberapa
cara. Cryptococcus diduga diperoleh melalui menghirup tanah yang terkontaminasi
dengan kotoran burung, dan Histoplasma ditemukan di lingkungan dengan
kontaminasi berat burung atau kotoran kelelawar, terutama di Midwest dekat Ohio
dan Mississippi Rivers. Blastomyces diduga ada di tanah kaya bahan organik yang
membusuk di Midwest Amerika Serikat, khususnya Midwest
utara. Coccidioidesditemukan di tanah daerah endemis (Barat AS dan bagian dari
Amerika Tengah dan Selatan). Ketika lingkungan ini terganggu, spora jamur dapat
terhirup.Hasil Meningitis dari infeksi jamur menyebar ke sumsum tulang
belakang. Candida biasanya diperoleh di rumah sakit.
Faktor Risiko
Penyakit tertentu, obat-obatan, dan prosedur bedah dapat melemahkan sistem
kekebalan tubuh dan meningkatkan risiko terkena infeksi jamur, yang dapat
menyebabkan meningitis jamur. Bayi prematur dengan berat lahir sangat rendah
juga pada peningkatan risiko untuk mendapatkanCandida infeksi aliran darah, yang
dapat menyebar ke otak.
Tinggal di daerah-daerah tertentu dari Amerika Serikat dapat meningkatkan risiko
untuk infeksi paru-paru jamur, yang juga bisa menyebar ke otak. Sebagai contoh,
burung dan kotoran kelelawar di Amerika Serikat Midwestern mungkin
berisi Histoplasma , dan tanah di Barat Daya Amerika Serikat mungkin
berisi Coccidioides .
Afrika Amerika, Filipina, wanita hamil pada trimester ketiga, dan orang-orang
dengan sistem kekebalan yang lemah lebih mungkin untuk
mendapatkan Coccidiodes infeksi, yang juga disebut demam lembah.

Tanda & Gejala

Tanda dan gejala meningitis jamur mungkin termasuk yang berikut:
 Demam
 Sakit kepala
 Kaku leher
 Mual dan muntah
 Fotofobia (kepekaan terhadap cahaya)
 Perubahan status mental
Diagnosa
Jika meningitis dicurigai, sampel darah atau cairan serebrospinal (dekat sumsum
tulang belakang) dikumpulkan dan dikirim ke laboratorium untuk
pengujian. Mengetahui penyebab spesifik dari meningitis adalah penting karena
tingkat keparahan penyakit dan pengobatan akan berbeda tergantung pada
penyebabnya.
Untuk mengkonfirmasi meningitis jamur, tes laboratorium tertentu dapat dilakukan,
tergantung pada jenis jamur yang dicurigai.
Pengobatan
Meningitis jamur diobati dengan kursus panjang dosis tinggi obat anti jamur,
biasanya diberikan melalui infus di rumah sakit. Panjang pengobatan tergantung
pada status sistem kekebalan tubuh dan jenis jamur yang menyebabkan
infeksi. Bagi orang-orang dengan sistem kekebalan yang tidak berfungsi dengan
baik karena kondisi lain, seperti AIDS, diabetes, atau kanker, pengobatan sering
lagi.
Pencegahan
Tidak ada kegiatan khusus yang diketahui menyebabkan meningitis jamur. Hindari
tanah dan lingkungan lainnya yang mungkin mengandung jamur. Orang dengan
sistem kekebalan yang lemah (misalnya, orang-orang dengan infeksi HIV) harus
mencoba untuk menghindari kotoran burung dan menghindari penggalian dan
kegiatan berdebu, terutama jika mereka tinggal di wilayah geografis di mana jamur
seperti Histoplasma , Coccidioides , atau Blastomyces spesies ada. Orang yang
terinfeksi HIV tidak dapat sepenuhnya menghindari paparan.

Who gets infected with Cryptococcus neoformans?

C. neoformans infections are more common among people with weakened immune
systems, but on very rare occasions, they can occur in people who are otherwise
healthy. Risk factors for infection with C. neoformans include:
 HIV /AIDS
 Corticosteroids and other immunosuppressive medications
 Solid organ transplantation
 Heart, lung, or liver diseases
 Diabetes
 Pregnancy

CSF findings in different forms of meningitis[26]

Type of meningitis Glucose Protein Cells

PMNs,
Acute bacterial low high
often > 300/mm³

mononuclear,
Acute viral normal normal or high
< 300/mm³

mononuclear and
Tuberculous low high
PMNs, < 300/mm³

Fungal low high < 300/mm³

usually
Malignant low high
mononuclear
Treatment and Outcomes for Cryptococcosis

Cryptococcosis requires treatment with prescription antifungal medication for at

least 6 months, usually longer. Treatment of severe cryptococcal infections,
including those with central nervous system involvement, usually begins with
amphotericin B, often in combination with flucytosine. For patients with
asymptomatic or mild-to-moderate cryptococcosis, fluconazole or itraconazole are
the preferred forms of treatment. Fluconazole can also be used for maintenance
therapy in HIV-infected patients with cryptococcal meningoencephalitis.